ICD-10-CM 2027
ICD-10 code for Birt-Hogg-Dube syndrome
Other specified congenital malformation syndromes, not elsewhere classified
From the official ICD-10-CM alphabetic index entry “Birt-Hogg-Dube syndrome”. Page updated September 29, 2026.
About coding Birt-Hogg-Dube syndrome
The ICD-10-CM code for Birt-Hogg-Dube syndrome is Q87.89 (Other specified congenital malformation syndromes, not elsewhere classified).
Within Q87.8, choose Q87.89 (Other specified congenital malformation syndromes, not elsewhere classified) only when documentation doesn't support a more specific option: Alport syndrome (Q87.81), arterial tortuosity syndrome (Q87.82), Bardet-Biedl syndrome (Q87.83), Laurence-Moon syndrome (Q87.84), MED13L syndrome (Q87.85), Kleefstra syndrome (Q87.86), Hao-Fountain Syndrome (Q87.87) and CTNNB1 syndrome (Q87.88).
Use an additional code(s) to identify all associated manifestations.
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
Common questions about Birt-Hogg-Dube syndrome ICD-10 codes
What is the ICD-10 code for Birt-Hogg-Dube syndrome?
- Q87.89 — Other specified congenital malformation syndromes, not elsewhere classified.
Is Q87.89 billable?
- Yes. Q87.89 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can Q87.89 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict Q87.89 as a principal diagnosis.
Is Q87.89 a CC or MCC?
- Q87.89 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.