2026 ICD-10-CM Diagnosis Code Q38.0Congenital malformations of lips, not elsewhere classified
ICD-10-CM Codes›Q00-Q99›Q38-Q45›Q38
- Billable — Valid for Submission
- POA Exempt
- Chronic Condition
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 11 through 13, 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
Code Classification
Present on Admission (POA)Billing
Q38.0 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Other congenital malformations of the digestive system (Q38-Q45).
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
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.
Malformation (congenital) See Also: Anomaly;
lip Q38.0
Van der Woude's syndrome Q38.0
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
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?
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Read the full article at MedlinePlus
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Convert Q38.0 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About Q38.0Overview
What is the ICD-10 code for congenital malformations of lips, not elsewhere classified?
The ICD-10-CM code for congenital malformations of lips, not elsewhere classified is Q38.0 (sometimes written as Q380). It is billable on HIPAA-covered claims from October 1, 2025 through September 30, 2026.
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 11, 12, 13, 157, 158, 159, with relative weights from 0.7085 to 5.4541 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. The code appears in the Other congenital malformations of the digestive system (Q38-Q45) range of the CMS exempt list.
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.
