2026 ICD-10-CM Diagnosis Code K00.0Anodontia
ICD-10-CM Codes›K00–K95›K00-K14›K00
- Billable — Valid for Submission
- Chronic Condition
K00.0 is a billable ICD-10-CM diagnosis code for anodontia. 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
Code Classification
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- 4H leukodystrophy
- Absence of teeth
- Anodontia
- Anodontia of permanent dentition
- Anodontia of primary dentition
- Cleft palate with stapes fixation and oligodontia syndrome
- Congenital absence of one tooth
- Congenital fusion of ossicles of ear
- Deafness and oligodontia syndrome
- Dental arch length loss
- Dental arch length loss due to congenitally missing teeth
- Developmental abnormality of deciduous tooth
- Developmental absence of tooth
- Epidermolysis bullosa simplex with hypodontia
- Familial hypodontia
- Hypodontia and nail dysgenesis
- Late tooth eruption
- Leukoencephalopathy, ataxia, hypodontia, hypomyelination syndrome
- Missing dentition by tooth number
- Odontoleukodystrophy
- Oligodontia
- Partial congenital absence of teeth
- Taurodontia with absent teeth and sparse hair syndrome
- Tooth absent
- X-linked hypodontia
- X-linked oligodontia
Tabular List NotesGuidance
Coding notes and annotation back-references applicable to this code.
Inclusion Terms
- Hypodontia
- Oligodontia
Type 1 Excludes
- acquired absence of teeth K08.1
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
- Anodontia (complete) (partial) (vera) - K00.0
- tooth bud - K00.0
External Cause of Injuries IndexGuidance
References for this code in the External Cause of Injuries Index.
- Absence(of) (organ or part) (complete or partial)
- teeth, tooth (congenital)
- Anodontia(complete) (partial) (vera)
- Deficiency, deficient
- tooth bud
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Anodontia
congenital absence of the teeth. it may involve all (total anodontia) or only some of the teeth (partial anodontia, hypodontia), or six or more of the teeth (oligodontia) and both the deciduous and the permanent dentition, or only teeth of the permanent dentition.Anodontia
the congenital absence of all teeth.AXIN2-Associated Polyposis|ODCRCS|Oligodontia-Colorectal Cancer Syndrome
a rare autosomal dominant syndrome caused by constitutional (germline) loss-of-function variants in axin2 gene. it is characterized by the presence of multiple colorectal adenomatous polyps and an increased risk of colorectal carcinoma. oligodontia and ectodermal dysplasia may or may not be present.EDA wt Allele|ECTD1|ED1|ED1-A1|ED1-A2|EDA|EDA-A1|EDA-A2|EDA1|EDA2|Ectodermal Dysplasia 1, Anhidrotic Gene|Ectodysplasin A wt Allele|Ectodysplasin Gene|HED|HED1|ODT1|Oligodontia 1 Gene|STHAGX1|TNLG7C|XHED|XLHED
human eda wild-type allele is located in the vicinity of xq13.1 and is approximately 423 kb in length. this allele, which encodes ectodysplasin-a protein, is involved in the morphogenesis of ectodermally derived tissues. mutation of the gene is associated with x-linked hypohidrotic ectodermal dysplasia type 1 and x-linked, selective tooth agenesis type 1.Oligodontia
the congenital absence of six or more permanent teeth with the exclusion of third molars.
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 K00.0 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About K00.0Overview
Is K00.0 (Disorders of tooth development and eruption) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report anodontia on HIPAA-covered claims from October 1, 2025 through September 30, 2026.
What MS-DRG does K00.0 group to?
When anodontia 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 K00.0?
Under the General Equivalence Mappings, anodontia converts to ICD-9-CM 520.0 (anodontia). The mapping is a direct match.
Footnotes
[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:
- The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
- The condition places limitations on self-care, independent living, and social interactions.
