2026 ICD-10-CM Diagnosis Code K00.5Hereditary disturbances in tooth structure, not elsewhere classified

ICD-10-CM Codes›K00–K95›K00-K14›K00

ICD-10-CM K00.5
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

K00.5 is a billable ICD-10-CM diagnosis code for hereditary disturbances in tooth structure, not elsewhere classified. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 157 through 159. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Any dental condition including traumatic injury, Disorders of teeth and gingiva, and Nontraumatic dental conditions.

Code Identity

ICD-10-CM Code
K00.5
Billable Status
Yes — Valid for Submission
Code Describes
Hereditary disturbances in tooth structure, not elsewhere classified
Short Description
Hereditary disturbances in tooth structure, NEC
Parent Code
K00
Disorders of tooth development and eruption

Code Classification

ChapterK00–K95Diseases of the digestive system
SectionK00-K14Diseases of oral cavity and salivary glands
CategoryK00Disorders of tooth development and eruption
This CodeK00.5Hereditary disturbances in tooth structure, not elsewhere classified

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Amelogenesis imperfecta
  • Amelogenesis imperfecta - hypomaturation - recessive pigmented
  • Amelogenesis imperfecta - hypomaturation - snow capped teeth
  • Amelogenesis imperfecta - hypoplastic autosomal dominant - local
  • Amelogenesis imperfecta - hypoplastic autosomal dominant - rough
  • Amelogenesis imperfecta - hypoplastic autosomal dominant - smooth
  • Amelogenesis imperfecta - recessive - rough
  • Amelogenesis imperfecta and gingival hyperplasia syndrome
  • Amelogenesis imperfecta co-occurrent with cone rod dystrophy
  • Amelogenesis imperfecta, hypocalcification type
  • Amelogenesis imperfecta, hypomaturation hypoplasia type with taurodontism
  • Amelogenesis imperfecta, hypomaturation type
  • Amelogenesis imperfecta, hypoplastic type
  • Amelogenesis imperfecta, hypoplastic type with microdontia
  • Amelogenesis imperfecta, pigmented hypomaturation type
  • Atypical dentin dysplasia due to SMOC2 deficiency
  • Brachyolmia
  • Congenital anomaly of sclera
  • Deep occlusal groove
  • Dentin dysplasia
  • Dentin dysplasia with sclerotic bone syndrome
  • Dentin dysplasia, type I
  • Dentin dysplasia, type II
  • Dentinogenesis imperfecta
  • Dentinogenesis imperfecta - Shield's type I
  • Dentinogenesis imperfecta - Shield's type II
  • Dentinogenesis imperfecta - Shield's type III
  • Dentinogenesis imperfecta, short stature, hearing loss, intellectual disability syndrome
  • Dermo-odonto dysplasia
  • Enamel-renal syndrome
  • Goldblatt syndrome
  • Horner's teeth
  • Hyperplasia of gingiva
  • Hypocalcification of teeth
  • Hypomineralization of enamel of teeth
  • Hypomineralization of enamel of tooth
  • Microdontia
  • Odontogenesis imperfecta
  • Osteogenesis imperfecta type I
  • Osteogenesis imperfecta with blue sclerae AND dentinogenesis imperfecta
  • Osteosclerosis
  • Shell teeth
  • Skeletal dysplasia with wormian bone, multiple fractures, dentinogenesis imperfecta syndrome
  • Taurodontism
  • Trichodysplasia with amelogenesis imperfecta syndrome
  • Verloes Bourguignon syndrome

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Amelogenesis imperfecta
  • Dentinogenesis imperfecta
  • Odontogenesis imperfecta
  • Dentinal dysplasia
  • Shell teeth

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Amelogenesis imperfecta
    • Brown enamel of teeth(hereditary)
    • Defect, defective
      • dentin (hereditary)
    • Dentin
      • opalescent
    • Dentinogenesis imperfecta
    • Disturbance(s)
      • tooth
        • structure, hereditary NEC
    • Dysplasia
      • dentinal
    • Odontogenesis imperfecta
    • Opalescent dentin(hereditary)
    • Shell teeth

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR DEN001
Any dental condition including traumatic injury
Default principal diagnosis: inpatient No · outpatient No
CCSR DIG002
Disorders of teeth and gingiva
Default principal diagnosis: inpatient Yes · outpatient Yes
CCSR DEN002
Nontraumatic dental conditions
Default principal diagnosis: inpatient No · outpatient No

Clinical InformationClinical

  • Dental Pulp Calcification

    calcinosis of the dental pulp or root canal.

  • Dentin Dysplasia

    an apparently hereditary disorder of dentin formation, marked by a normal appearance of coronal dentin associated with pulpal obliteration, faulty root formation, and a tendency for peripheral lesions without obvious cause. (from dorland, 27th ed)

  • Dentinogenesis Imperfecta

    an autosomal dominant disorder of tooth development characterized by opalescent dentin resulting in discoloration of the teeth. the dentin develops poorly with low mineral content while the pulp canal is obliterated.

  • Osteopetrosis

    excessive formation of dense trabecular bone leading to pathological fractures; osteitis; splenomegaly with infarct; anemia; and extramedullary hemopoiesis (hematopoiesis, extramedullary).

  • Osteosclerosis

    an abnormal hardening or increased density of bone tissue.

  • Amelogenesis Imperfecta

    a clinically and genetically heterogeneous group of hereditary conditions characterized by malformed dental enamel, usually involving dental enamel hypoplasia and/or tooth hypomineralization.

  • COL1A2 wt Allele|COL1A2|Collagen Type I Alpha 2 Chain wt Allele|Collagen of Skin, Tendon and Bone, Alpha-2 Chain Gene|Collagen, Type I, Alpha 2 Gene|Collagen, Type I, Alpha-2 Gene|EDSARTH2|EDSCV|OI4|Osteogenesis Imperfecta Type IV Gene

    human col1a2 wild-type allele is located in the vicinity of 7q22.1 and is approximately 37 kb in length. this allele, which encodes collagen alpha-2 (i) chain protein, plays a role in the structural integrity of tendons, ligaments and bones. mutations in the gene are associated with atypical marfan syndrome, ehlers-danlos syndrome types and osteogenesis imperfecta types.

  • Dentinogenesis Imperfecta

    a congenital tooth development disorder caused by mutations in the dspp gene. the teeth are weak, discolored, and translucent.

  • COL1A2 wt Allele|COL1A2|Collagen Type I Alpha 2 Chain wt Allele|Collagen, Type I, Alpha 2 Gene|OI4|Osteogenesis Imperfecta Type IV Gene

    human col1a2 wild-type allele is located in the vicinity of 7q22.1 and is approximately 37 kb in length. this allele, which encodes collagen alpha-2 (i) chain protein, plays a role in the structural integrity of tendons, ligaments and bones. mutations in the gene are associated with atypical marfan syndrome, ehlers-danlos syndrome types and osteogenesis imperfecta types.

  • Osteogenesis Imperfecta Type I

    the mildest and most common type of osteogenesis imperfecta. it is characterized by bone fractures, muscle weakness, and loose joints. bone deformities are either absent or minimal.

  • Osteogenesis Imperfecta Type II

    a severe form of osteogenesis imperfecta. it is characterized by bone deformities, multiple fractures, underdeveloped lungs, and often death during or after birth due to respiratory abnormalities.

  • Osteogenesis Imperfecta Type III

    a type of osteogenesis imperfecta characterized by bone fractures, bone deformities, short stature, poor muscle development, barrel-shaped chest, and triangular face.

  • Osteogenesis Imperfecta Type IV

    a type of osteogenesis imperfecta that is characterized by fractures and hearing loss. it is more severe than type i and less severe than types ii and iii.

  • Nephrocalcinosis

    deposition of calcium in the renal parenchyma, resulting from high levels of calcium in the blood and/or urine.

  • Osteosclerosis

    abnormally high bone density.

Patient EducationClinical

Tooth Disorders

Your teeth are made of a hard, bonelike material. There are four parts:

The full article covers:

  • What are teeth?
  • What are tooth disorders?
  • What causes tooth disorders?
  • What are the symptoms of tooth disorders?
  • How are tooth disorders diagnosed?
  • What are the treatments for tooth disorders?
  • Can tooth disorders be prevented?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert K00.5 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
520.5 Heredit tooth struct NEC
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About K00.5Overview

Is K00.5 (Disorders of tooth development and eruption) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report hereditary disturbances in tooth structure, not elsewhere classified on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does K00.5 group to?

When hereditary disturbances in tooth structure, not elsewhere classified is the principal diagnosis on an inpatient stay, it groups to MS-DRG 157, 158, 159, with relative weights from 0.7085 to 1.7168 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of K00.5?

Under the General Equivalence Mappings, hereditary disturbances in tooth structure, not elsewhere classified converts to ICD-9-CM 520.5 (heredit tooth struct NEC). The mapping is a direct match.

Footnotes

[1] Not chronic - A diagnosis code that does not fit the criteria for chronic condition (duration, ongoing medical treatment, and limitations) is considered not chronic. Some codes designated as not chronic are acute conditions. Other diagnosis codes that indicate a possible chronic condition, but for which the duration of the illness is not specified in the code description (i.e., we do not know the condition has lasted 12 months or longer) also are considered not chronic.