ICD-10-CM 2027
ICD-10 code for Kabuki syndrome
From the official ICD-10-CM alphabetic index entry “Syndrome › Kabuki (type 1, due to KMT2D mutation) (type 2, due to KDM6A mutation)”. Page updated September 29, 2026.
About coding Kabuki syndrome
The ICD-10-CM code for Kabuki syndrome is Q89.81 (Kabuki syndrome).
Within Q89.8 (other specified congenital malformations), Q89.81 is specifically for Kabuki syndrome. Related codes cover other specified congenital malformations (Q89.89).
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.
In MS-DRG v44.0, it is part of the grouping logic for DRG 564 (Other Musculoskeletal System and Connective Tissue Diagnoses with MCC, relative weight 1.4908), DRG 565 (Other Musculoskeletal System and Connective Tissue Diagnoses with CC, relative weight 0.9783) and DRG 566 (Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCC, relative weight 0.7378), in MDC 08 (Diseases and Disorders of the Musculoskeletal System and Connective Tissue), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Kabuki syndrome ICD-10 codes
What is the ICD-10 code for Kabuki syndrome?
- Q89.81 — Kabuki syndrome.
Is Q89.81 billable?
- Yes. Q89.81 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can Q89.81 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict Q89.81 as a principal diagnosis.
Is Q89.81 a CC or MCC?
- Q89.81 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.