Q89.81 Kabuki syndrome

The ICD-10-CM code for Kabuki syndrome is Q89.81 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

17: Congenital malformations, deformations and chromosomal abnormalities (Q00-QA0)

Section

Q80-Q89: Other congenital malformations (Q80-Q89)

Category Q89

13 codes (10 billable)

FY2026 Status

New code in FY2026

Also Known As

ICD-10-CM Alphabetic Index entries that lead to Q89.81:

• Syndrome › Kabuki (type 1, due to KMT2D mutation) (type 2, due to KDM6A mutation)
• Syndrome › Niikawa-Kuroki

Inclusion Terms

• Kabuki syndrome, type 1, due to KMT2D mutation
• Kabuki syndrome, type 2, due to KDM6A mutation
• Niikawa-Kuroki syndrome

Official Coding Guidelines

POA indicator for congenital conditions/anomalies and Q00-Q99 exception

Congenital conditions and anomalies Assign “Y” for congenital conditions and anomalies except for categories Q00- Q99, Congenital anomalies, which are on the exempt list. Congenital conditions are always considered present on admission.

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool