2026 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 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q44.6 is a billable ICD-10-CM diagnosis code for cystic disease of liver. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 441 through 443. 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 other POA exempt codes.

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

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Fibrocystic disease of liver

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Cyst(colloid) (mucous) (simple) (retention)
      • congenital NEC
        • liver
    • Cyst(colloid) (mucous) (simple) (retention)
      • liver (idiopathic) (simple)
        • congenital
    • Cystic
      • liver, congenital
    • Degeneration, degenerative
      • liver (diffuse) NEC
        • cystic
          • congenital
    • Disease, diseased
      • cystic
        • liver, congenital
    • Disease, diseased
      • liver (chronic) (organic)
        • cystic, congenital
    • Disease, diseased
      • liver (chronic) (organic)
        • fibrocystic (congenital)
    • Disease, diseased
      • liver (chronic) (organic)
        • polycystic (congenital)
    • Disease, diseased
      • polycystic
        • liver or hepatic
    • Fibrocystic
      • disease
        • liver
    • Polycystic(disease)
      • liver

Clinical ClassificationClinical

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

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

Clinical InformationClinical

  • Choledochal Cyst

    cystic dilatation of the hepatic duct or bile duct.

  • Congenital Hepatic Fibrosis

    a congenital disorder usually inherited in an autosomal recessive pattern. it affects the hepatobiliary system and the kidneys. it is characterized by liver fibrosis, portal hypertension, and renal cysts.

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?
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  • 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 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About Q44.6Overview

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, 2025 through September 30, 2026.

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 441, 442, 443, with relative weights from 0.6997 to 1.7947 depending on complications. Higher weights mean higher Medicare reimbursement.

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.

What is the ICD-9 equivalent of Q44.6?

Under the General Equivalence Mappings, cystic disease of liver converts to ICD-9-CM 751.62 (cong cystic liver dis). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.