2026 ICD-10-CM Diagnosis Code Q63.9Congenital malformation of kidney, unspecified

ICD-10-CM Codes›Q00-Q99›Q60-Q64›Q63

ICD-10-CM Q63.9
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q63.9 is a billable ICD-10-CM diagnosis code for congenital malformation of kidney, unspecified. 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
Q63.9
Billable Status
Yes — Valid for Submission
Code Describes
Congenital malformation of kidney, unspecified
Short Description
Congenital malformation of kidney, unspecified
Same as the full description in the CMS dataset.
Parent Code
Q63
Other congenital malformations of kidney

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ60-Q64Congenital malformations of the urinary system
CategoryQ63Other congenital malformations of kidney
This CodeQ63.9Congenital malformation of kidney, unspecified

Present on Admission (POA)Billing

Q63.9 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.

  • Acrorenal mandibular syndrome
  • Acrorenal syndrome
  • Acrorenoocular syndrome
  • Anal atresia
  • Cardiomyopathy and renal anomaly syndrome
  • Congenital anomaly of renal pelvis
  • Congenital anomaly of the kidney
  • Congenital anomaly of ureter and renal pelvis
  • Congenital fusion of kidneys
  • Congenital hypoplasia of bone of radius and/or ulna
  • Congenital hypoplasia of fibula
  • Congenital hypoplasia of ulna
  • Congenital vertebral, cardiac, renal anomalies syndrome
  • Defect of vertebral segmentation
  • Faciocardiorenal syndrome
  • Familial aplasia of the vermis
  • Horseshoe kidney
  • Hypotrichosis, lymphedema, telangiectasia, renal defect syndrome
  • Ichthyosis, intellectual disability, dwarfism, renal impairment syndrome
  • Joubert syndrome
  • Joubert syndrome with oculorenal defect
  • Mitochondrial DNA depletion syndrome hepatocerebrorenal form
  • Nephronophthisis
  • Neurofaciodigitorenal syndrome
  • Radio-renal syndrome
  • Saito Kuba Tsuruta syndrome
  • SERKAL syndrome
  • STAR syndrome
  • Tall stature, intellectual disability, renal anomalies syndrome
  • Telecanthus
  • VACTERL syndrome with hydrocephalus
  • Vertebral abnormalities, anal atresia, cardiac abnormalities, tracheo-esophageal fistula, renal anomalies, limb defects syndrome

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.

    • Anomaly, anomalous(congenital) (unspecified type)
      • kidney (s) (calyx) (pelvis)
    • Anomaly, anomalous(congenital) (unspecified type)
      • renal
    • Anomaly, anomalous(congenital) (unspecified type)
      • renal
        • pelvis
    • Deformity
      • kidney (s) (calyx) (pelvis) (congenital)
    • Malformation(congenital)
      • kidney

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

  • 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.

  • Horseshoe Kidney

    a congenital abnormality in which the two kidneys fuse together during fetal development to create a horseshoe-shaped structure.

  • Joubert Syndrome

    a rare genetic syndrome characterized by the hypoplasia or absence of the cerebellar vermis. signs and symptoms include rapid breathing (hyperpnea), sleep apnea, abnormal eye movements, mental retardation, and ataxia.

  • Joubert Syndrome 17|JBTS17

    an autosomal recessive subtype of joubert syndrome caused by mutation(s) in the cplane1 gene, encoding ciliogenesis and planar polarity effector 1.

  • Joubert Syndrome 3|JBTS3

    an autosomal recessive subtype of joubert syndrome caused by mutation(s) in the ahi1 gene, encoding jouberin.

  • Joubert Syndrome 4

    a rare genetic syndrome caused by mutations in the nphp1 gene. it is characterized by the hypoplasia or absence of the cerebellar vermis. signs and symptoms include rapid breathing (hyperpnea), sleep apnea, abnormal eye movements, mental retardation, and ataxia.

  • Joubert Syndrome 7|JBTS7

    an autosomal recessive sub-type of joubert syndrome caused by mutation(s) in the rpgrip1l gene, encoding a protein thought to function in programmed cell death. it is characterized by cerebellar and oculomotor apraxia, hypotonia and psychomotor delay, neonatal respiratory abnormalities, renal abnormalities, and retinal dystrophy.

  • Joubert Syndrome 9|JBTS9

    an autosomal recessive subtype of joubert syndrome caused by mutation(s) in the cc2d2a gene, encoding coiled-coil and c2 domain-containing protein 2a.

Patient EducationClinical

Kidney Diseases

You have two kidneys, each about the size of your fist. They are near the middle of your back, just below the rib cage. Inside each kidney there are about a million tiny structures called nephrons. They filter your blood. They remove wastes and extra water, which become urine. The urine flows through tubes called ureters.

Read the full article at MedlinePlus

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

Convert Q63.9 to ICD-9-CMHistory

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

ICD-9-CM
753.3 Kidney anomaly NEC
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 Q63.9Overview

Is Q63.9 (Other congenital malformations of kidney) a billable code?

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

What MS-DRG does Q63.9 group to?

When congenital malformation of kidney, unspecified 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 Q63.9 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 congenital malformation of kidney, unspecified on inpatient claims.

What is the ICD-9 equivalent of Q63.9?

Under the General Equivalence Mappings, congenital malformation of kidney, unspecified converts to ICD-9-CM 753.3 (kidney anomaly NEC). 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.