2027 ICD-10-CM Diagnosis Code Q89.81Kabuki syndrome

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q89

ICD-10-CM Q89.81
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q89.81 is a billable ICD-10-CM diagnosis code for kabuki syndrome. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 564 through 566. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 40 closely related codes. The code is exempt from POA reporting. Coders also document this condition as kabuki make-up syndrome. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified congenital anomalies.

Code Identity

ICD-10-CM Code
Q89.81
Billable Status
Yes — Valid for Submission
Code Describes
Kabuki syndrome
Short Description
Kabuki syndrome
Same as the full description in the CMS dataset.
Parent Code
Q89.8
Other specified congenital malformations

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ89Other congenital malformations, not elsewhere classified
This CodeQ89.81Kabuki syndrome

Present on Admission (POA)Billing

Q89.81 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Other congenital malformations (Q80-Q89).

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Kabuki make-up syndrome

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q89.81: its own notes plus those printed at Q89.8 and Chapter 17. A note printed at a category, block or chapter applies to every code under it.

Applicable To

Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.

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

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • inborn errors of metabolism (E70-E88)

Use Additional Code

Add a second code, after this one, to fully describe the condition.

From Q89.8 Other specified congenital malformations applies to 2 codes
  • code(s) to identify all associated manifestations

Notes

General instructions on how these codes are used.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • Codes from this chapter are not for use on maternal records

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MAL010
Other specified and unspecified congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Kabuki Syndrome

    a rare, autosomal dominant or x-linked dominant inherited syndrome caused by mutations in the kmt2d gene (also known as mll2) or the kdm6a gene. it is characterized by distinctive facial features including arched eyebrows, long eyelashes, long palpebral fissures with the lower lids turned out at the outside edges, a flat nose, and large protruding earlobes, developmental delay and intellectual disability.

Code History & ChangesHistory

Replacement Q89.81 replaces the following previously assigned code(s):

  • Q89.8 - Other specified congenital malformations
FY 2026AddedAdded to the ICD-10-CM code setEffective October 1, 2025.
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q89.81Overview

What is the ICD-10 code for kabuki syndrome?

The ICD-10-CM code for kabuki syndrome is Q89.81 (sometimes written as Q8981). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is Q89.81 (Other specified congenital malformations) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report kabuki syndrome on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does Q89.81 group to?

When kabuki syndrome is the principal diagnosis on an inpatient stay, it groups to MS-DRG 564, 566, 565, with relative weights from 0.7374 to 1.4909 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q89.81 a CC or MCC?

CMS lists Q89.81 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 40 closely related codes in its exclusion list.

Is Q89.81 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 kabuki syndrome on inpatient claims. The code appears in the Other congenital malformations (Q80-Q89) range of the CMS exempt list.