2026 ICD-10-CM Diagnosis Code Q61.5Medullary cystic kidney

ICD-10-CM Codes›Q00-Q99›Q60-Q64›Q61

ICD-10-CM Q61.5
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q61.5 is a billable ICD-10-CM diagnosis code for medullary cystic kidney. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 698 through 700. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Genitourinary congenital anomalies.

Code Identity

ICD-10-CM Code
Q61.5
Billable Status
Yes — Valid for Submission
Code Describes
Medullary cystic kidney
Short Description
Medullary cystic kidney
Same as the full description in the CMS dataset.
Parent Code
Q61
Cystic kidney disease

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ60-Q64Congenital malformations of the urinary system
CategoryQ61Cystic kidney disease
This CodeQ61.5Medullary cystic kidney

Present on Admission (POA)Billing

Q61.5 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.

  • Adolescent nephronophthisis
  • Adult familial nephronophthisis with spastic quadriparesia syndrome
  • Autosomal dominant tubulointerstitial kidney disease
  • Bilateral medullary sponge kidney
  • Boichis syndrome
  • Congenital hepatic fibrosis
  • Fibrocystic kidney disease
  • Infantile nephronophthisis
  • Juvenile nephronophthisis
  • Medullary cystic disease of the kidney
  • Medullary cystic disease, adult type
  • Medullary sponge kidney
  • Medullary sponge kidney with nephrocalcinosis
  • Medullary sponge kidney without nephrocalcinosis
  • MUC1-related autosomal dominant tubulointerstitial kidney disease
  • Nephronophthisis
  • Nephronophthisis - medullary cystic disease
  • Nephronophthisis type 4
  • Nephronophthisis type 5
  • Nephronophthisis type 6
  • Paresis of left lower limb
  • Paresis of right lower limb
  • RHYNS syndrome
  • Spastic tetraparesis
  • Tetraparesis
  • UMOD-related autosomal dominant tubulointerstitial kidney disease
  • Weakness of bilateral lower limb
  • Weakness of left lower limb
  • Weakness of left upper limb
  • Weakness of right lower limb
  • Weakness of right upper limb

Tabular List NotesGuidance

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

Inclusion Terms

  • Nephronophthisis
  • Sponge kidney NOS

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.

    • Cystic
      • kidney (congenital)
        • medullary
    • Cystic
      • medullary, kidney
    • Medullary cystic kidney
    • Nephronophthisis
    • Sponge
      • kidney (medullary)

Clinical ClassificationClinical

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

CCSR MAL003
Genitourinary congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Medullary Sponge Kidney

    a non-hereditary kidney disorder characterized by the abnormally dilated (ectasia) medullary and inner papillary portions of the collecting ducts. these collecting ducts usually contain cysts or diverticula filled with jelly-like material or small calculi (kidney stones) leading to infections or obstruction. it should be distinguished from congenital or hereditary polycystic kidney diseases.

  • Nephrocystin-1|Juvenile Nephronophthisis 1 Protein|NPHP1

    nephrocystin-1 (732 aa, ~83 kda) is encoded by the human nphp1 gene. this protein is involved in the modulation of signaling.

  • Nephronophthisis

    progressive tubulointerstitial injury, inherited in an autosomal recessive pattern, caused by mutations in genes involved in ciliary function, which may result in an end stage renal failure.

  • Nephronophthisis 1|Familial Juvenile Nephronophthisis|Juvenile Nephronophthisis|NPH1

    progressive tubulointerstitial nephritis inherited in an autosomal recessive manner. it is caused by mutations in the nphp1 gene. patients present with anemia, polyuria, and polydipsia during childhood. the progressive bilateral kidney damage results in renal failure.

  • NPHP1 Gene|NPHP1|NPHP1|Nephronophthisis 1 (Juvenile) Gene

    this gene is involved in the mediation of signal transduction.

  • NPHP1 wt Allele|FLJ97602|JBTS4|NPH1|Nephronophthisis 1 (Juvenile) wt Allele|SLSN1

    human nphp1 wild-type allele is located in the vicinity of 2q13 and is approximately 83 kb in length. this allele, which encodes nephrocystin-1 protein, plays a role in the progression of adhesion-dependent signaling pathways. mutations in the gene are associated with familial juvenile nephronophthisis type 1, senior-loken syndrome type 1, and joubert syndrome type 4.

  • Medullary Sponge Kidney

    a developmental disorder of the kidney characterized by cystic dilatation of the medullary collecting ducts, resulting in a spongy gross appearance of the kidney. it may be asymptomatic or complicated by hematuria, infections, or renal stones.

  • 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

Kidney Cysts

A cyst is a fluid-filled sac. You may get simple kidney cysts as you age; they are usually harmless. There are also some diseases which cause kidney cysts. One type is polycystic kidney disease (PKD). It runs in families. In PKD, many cysts grow in the kidneys. This can enlarge the kidneys and make them work poorly.

Read the full article at MedlinePlus

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

Convert Q61.5 to ICD-9-CMHistory

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

ICD-9-CM
753.16 Medullary cystic kidney
Approximate The match is approximate rather than exact.
ICD-9-CM
753.17 Medullary sponge kidney
Approximate The match is approximate rather than exact.

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 Q61.5Overview

Is Q61.5 (Cystic kidney disease) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report medullary cystic kidney on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does Q61.5 group to?

When medullary cystic kidney is the principal diagnosis on an inpatient stay, it groups to MS-DRG 698, 699, 700, with relative weights from 0.6899 to 1.6544 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q61.5 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 medullary cystic kidney on inpatient claims.

What is the ICD-9 equivalent of Q61.5?

Under the General Equivalence Mappings, medullary cystic kidney converts to ICD-9-CM 753.16 (medullary cystic kidney) and 753.17 (medullary sponge kidney). The mapping is approximate, so confirm the match fits the documentation.

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.