2026 ICD-10-CM Diagnosis Code Q38.0Congenital malformations of lips, not elsewhere classified

ICD-10-CM Codes›Q00-Q99›Q38-Q45›Q38

ICD-10-CM Q38.0
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q38.0 is a billable ICD-10-CM diagnosis code for congenital malformations of lips, 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. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Digestive congenital anomalies.

Code Identity

ICD-10-CM Code
Q38.0
Billable Status
Yes — Valid for Submission
Code Describes
Congenital malformations of lips, not elsewhere classified
Short Description
Congenital malformations of lips, not elsewhere classified
Same as the full description in the CMS dataset.
Parent Code
Q38
Other congenital malformations of tongue, mouth and pharynx

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ38-Q45Other congenital malformations of the digestive system
CategoryQ38Other congenital malformations of tongue, mouth and pharynx
This CodeQ38.0Congenital malformations of lips, not elsewhere classified

Present on Admission (POA)Billing

Q38.0 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review other POA exempt codes.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Aberrant insertion of labial frenulum
  • Broad attachment of labial frenum
  • Caliber persistent labial artery
  • Congenital anomaly of lip
  • Congenital commissural pits
  • Congenital double lip
  • Congenital ectropion of lip
  • Congenital fistula of commissure of lips
  • Congenital fistula of lip
  • Congenital hyperplasia of sebaceous glands of lip
  • Congenital lip pits
  • Congenital lower lip pits
  • Congenital midline raphe of philtrum
  • Congenital midline sinus of philtrum
  • Enlarged labial frenum
  • Finding of arrangement of lip
  • Fistula of lip
  • High upper lip line
  • Ichthyosis, alopecia, eclabion, ectropion, intellectual disability syndrome
  • Lamellar ichthyosis
  • Median nodule of upper lip
  • Sebaceous hyperplasia
  • Short philtrum
  • Synchilia
  • Van der Woude syndrome

Tabular List NotesGuidance

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

Inclusion Terms

  • Congenital fistula of lip
  • Congenital malformation of lip NOS
  • Van der Woude's syndrome

Type 1 Excludes

  • cleft lip Q36
  • cleft lip with cleft palate Q37
  • macrocheilia Q18.6
  • microcheilia Q18.7

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.

    • Agenesis
      • lip
    • Anomaly, anomalous(congenital) (unspecified type)
      • lip
    • Deformity
      • lip (acquired) NEC
        • congenital
    • Ectropion
      • lip (acquired)
        • congenital
    • Fistula(cutaneous)
      • lip
        • congenital
    • Malformation(congenital)
      • lip
    • Van der Woude's syndrome

Clinical ClassificationClinical

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

CCSR MAL002
Digestive congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • ABCA12 wt Allele|ABC12|ARCI4A|ARCI4B|ATP Binding Cassette Subfamily A Member 12 wt Allele|ATP-Binding Cassette, Sub-Family A (ABC1), Member 12 Gene|ATP-Binding Cassette, Subfamily A, Member 12 Gene|DKFZP434G232|ICR2B|Ichthyosis Congenita II, Lamellar Ichthyosis B Gene|LI2

    human abca12 wild-type allele is located in the vicinity of 2q35 and is approximately 207 kb in length. this allele, which encodes glucosylceramide transporter abca12 protein, plays a role in both the membrane localization of glucosylceramide and other lipids in lamellar granules and in cholesterol transport. mutation of the gene is associated with autosomal recessive congenital ichthyosis (arci) types 4a and 4b (harlequin).

  • Lamellar Ichthyosis

    a very rare, autosomal recessive inherited skin disorder present at birth. it is characterized by the presence of a transparent membrane encasing the newborn. this membrane sheds in about two weeks after birth to reveal generalized scaling and skin erythema.

  • Sebaceous Hyperplasia

    enlargement of the sebaceous glands.

  • Senile Sebaceous Hyperplasia

    a hyperplasia of the sebaceous glands. it is usually seen as small yellow papules in the face of middle aged-older population.

Patient EducationClinical

Birth Defects

A birth defect is a problem that happens while a baby is developing in the mother's body. Most birth defects happen during the first 3 months of pregnancy. One out of every 33 babies in the United States is born with a birth defect.

The full article covers:

  • What are birth defects?
  • What causes birth defects?
  • Who is at risk of having a baby with birth defects?
  • How are birth defects diagnosed?
  • What are the treatments for birth defects?
  • Can birth defects be prevented?

Read the full article at MedlinePlus

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

Convert Q38.0 to ICD-9-CMHistory

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

ICD-9-CM
750.25 Congenital lip fistula
Approximate The match is approximate rather than exact.

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 Q38.0Overview

Is Q38.0 (Other congenital malformations of tongue, mouth and pharynx) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report congenital malformations of lips, not elsewhere classified on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does Q38.0 group to?

When congenital malformations of lips, 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.

Is Q38.0 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for congenital malformations of lips, not elsewhere classified on inpatient claims.

What is the ICD-9 equivalent of Q38.0?

Under the General Equivalence Mappings, congenital malformations of lips, not elsewhere classified converts to ICD-9-CM 750.25 (congenital lip fistula). The mapping is approximate, so confirm the match fits the documentation.

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.