Q89.81 – Kabuki syndrome

ICD-10-CM 2027 diagnosis code · Other congenital malformations

Billable code

ICD-10-CM code Q89.81
Code
Q89.81 (claims format: Q8981)
Description
Kabuki syndrome
Billable
Yes – valid for HIPAA-covered transactions
Valid for
Dates of service October 1, 2026 – September 30, 2027 (FY2027)
Chapter
17. Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders (Q00-QA1)
Block
Q80-Q89 Other congenital malformations
Parent codes
Q89 › Q89.8

Notes for Q89.81

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

Notes inherited from parent codes

Instructional notes at a category or block level apply to every code below it.

Q89.8 – Other specified congenital malformations

Use additional code:
  • code(s) to identify all associated manifestations

Index terms for Q89.81

Entries in the ICD-10-CM alphabetic index that lead to this code:

Related codes in Q89.8

Frequently asked questions

What is ICD-10 code Q89.81?

Q89.81 is the ICD-10-CM code for kabuki syndrome, in the block Q80-Q89 (Other congenital malformations).

Is Q89.81 a billable code?

Yes. Q89.81 is a billable/specific code that can be used to indicate a diagnosis for reimbursement purposes.

What category does Q89.81 belong to?

It belongs to category Q89 – Other congenital malformations, not elsewhere classified.

Source: CDC/NCHS ICD-10-CM FY2027 code files, tabular list and index. Reference only – code assignment must follow the official ICD-10-CM guidelines and documentation in the medical record.