2026 ICD-10-CM Diagnosis Code E71.518Other disorders of peroxisome biogenesis

ICD-10-CM Codes›E00–E89›E70-E88›E71

ICD-10-CM E71.518
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E71.518 is a billable ICD-10-CM diagnosis code for other disorders of peroxisome biogenesis. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E71.518
Billable Status
Yes — Valid for Submission
Code Describes
Other disorders of peroxisome biogenesis
Short Description
Other disorders of peroxisome biogenesis
Same as the full description in the CMS dataset.
Parent Code
E71.51
Disorders of peroxisome biogenesis

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
This CodeE71.518Other disorders of peroxisome biogenesis

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Alpha-methylacyl-CoA racemase deficiency disorder
  • General loss of peroxisomal function
  • Peroxisome biogenesis disorder
  • PEX1 deficiency
  • PEX10 deficiency
  • PEX12 deficiency
  • PEX13 deficiency
  • PEX14 deficiency
  • PEX16 deficiency
  • PEX19 deficiency
  • PEX2 deficiency
  • PEX26 deficiency
  • PEX3 deficiency
  • PEX5 deficiency
  • PEX6 deficiency
  • Synthetic defect of bile acids

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.

    • Disorder(of)
      • peroxisomal
        • biogenesis
          • specified disorder NEC
    • Disorder(of)
      • peroxisomal
        • specified form NEC
          • group 1

Clinical ClassificationClinical

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

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Leukodystrophies

Leukodystrophies are a group of rare genetic disorders that affect the central nervous system (CNS). The CNS is made up of your brain and spinal cord. Leukodystrophies damage the white matter of your CNS. The white matter includes:

The full article covers:

  • What are leukodystrophies?
  • What causes leukodystrophies?
  • What are the symptoms of leukodystrophies?
  • How are leukodystrophies diagnosed?
  • What are the treatments for leukodystrophies?

Read the full article at MedlinePlus

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

Convert E71.518 to ICD-9-CMHistory

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

ICD-9-CM
277.86 Peroxisomal disorders
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 E71.518Overview

Is E71.518 (Disorders of peroxisome biogenesis) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report other disorders of peroxisome biogenesis on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E71.518?

Under the General Equivalence Mappings, other disorders of peroxisome biogenesis converts to ICD-9-CM 277.86 (peroxisomal disorders). 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.