2027 ICD-10-CM Diagnosis Code Q44.6Cystic disease of liver

ICD-10-CM Codes›Q00-Q99›Q38-Q45›Q44

ICD-10-CM Q44.6
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q44.6 is a billable ICD-10-CM diagnosis code for cystic disease of liver. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 441 through 443. 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 8 closely related codes. The code is exempt from POA reporting. Coders also document this condition as caroli syndrome. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Digestive congenital anomalies.

Code Identity

ICD-10-CM Code
Q44.6
Billable Status
Yes — Valid for Submission
Code Describes
Cystic disease of liver
Short Description
Cystic disease of liver
Same as the full description in the CMS dataset.
Parent Code
Q44
Congenital malformations of gallbladder, bile ducts and liver

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ38-Q45Other congenital malformations of the digestive system
CategoryQ44Congenital malformations of gallbladder, bile ducts and liver
This CodeQ44.6Cystic disease of liver

Present on Admission (POA)Billing

Q44.6 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Other congenital malformations of the digestive system (Q38-Q45).

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Caroli syndrome
  • Congenital cystic disease of liver
  • Congenital hepatic fibrosis
  • Cystic dilation of intrahepatic duct
  • Isolated polycystic liver disease
  • Liver cyst
  • Polycystic liver disease-3 due to heterozygous mutation of ALG8 gene

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q44.6: its own notes plus those printed at 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.

  • Fibrocystic disease of liver

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)

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.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name Q44.6, its category, or a range that includes it.

Excludes2 1

These codes carry an Excludes2 note naming Q44.6: its condition is not part of those codes, and both may be reported when the patient has both.

  • K76 Other diseases of liver
    cystic disease of liver (congenital) (Q44.6)

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 MAL002
Digestive congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Birth Defects

A birth defect is a problem that happens while a baby is developing in the mother's body. Most birth defects happen during the first 3 months of pregnancy. One out of every 33 babies in the United States is born with a birth defect.

The full article covers:

  • What are birth defects?
  • What causes birth defects?
  • Who is at risk of having a baby with birth defects?
  • How are birth defects diagnosed?
  • What are the treatments for birth defects?
  • Can birth defects be prevented?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert Q44.6 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
751.62 Cong cystic liver dis
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q44.6Overview

What is the ICD-10 code for cystic disease of liver?

The ICD-10-CM code for cystic disease of liver is Q44.6 (sometimes written as Q446). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is Q44.6 a billable code?

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

What MS-DRG does Q44.6 group to?

When cystic disease of liver is the principal diagnosis on an inpatient stay, it groups to MS-DRG 442, 441, 443, with relative weights from 0.6890 to 1.8226 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q44.6 a CC or MCC?

CMS lists Q44.6 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 8 closely related codes in its exclusion list.

Is Q44.6 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 cystic disease of liver on inpatient claims. The code appears in the Other congenital malformations of the digestive system (Q38-Q45) range of the CMS exempt list.