ICD-10-CM 2027
ICD-10 code for peroxisomal disorder
From the official ICD-10-CM alphabetic index entry “Disorder (of) › peroxisomal”. Page updated September 29, 2026.
About coding peroxisomal disorder
The ICD-10-CM code for peroxisomal disorder is E71.50 (Peroxisomal disorder, unspecified). The official index lists 14 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on E71.50.
Within E71.5, choose E71.50 (Peroxisomal disorder, unspecified) only when documentation doesn't support a more specific option: disorders of peroxisome biogenesis (E71.51), X-linked adrenoleukodystrophy (E71.52), other group 2 peroxisomal disorders (E71.53) and other peroxisomal disorders (E71.54).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
More specific peroxisomal disorder codes (official index)
- biogenesis
- neonatal adrenoleukodystrophy E71.511 — Neonatal adrenoleukodystrophy
- specified disorder NEC E71.518 — Other disorders of peroxisome biogenesis
- Zellweger syndrome E71.510 — Zellweger syndrome
- rhizomelic chondrodysplasia punctata E71.540 — Rhizomelic chondrodysplasia punctata
- specified form NEC E71.548 — Other peroxisomal disorders
- group 1 E71.518 — Other disorders of peroxisome biogenesis
- group 2 E71.53 — Other group 2 peroxisomal disorders
- group 3 E71.542 — Other group 3 peroxisomal disorders
- X-linked adrenoleukodystrophy E71.529 — X-linked adrenoleukodystrophy, unspecified type
- adolescent E71.521 — Adolescent X-linked adrenoleukodystrophy
- adrenomyeloneuropathy E71.522 — Adrenomyeloneuropathy
- childhood E71.520 — Childhood cerebral X-linked adrenoleukodystrophy
- specified form NEC E71.528 — Other X-linked adrenoleukodystrophy
- Zellweger-like syndrome E71.541 — Zellweger-like syndrome
Common questions about peroxisomal disorder ICD-10 codes
What is the ICD-10 code for peroxisomal disorder?
- E71.50 — Peroxisomal disorder, unspecified. More specific codes apply when the documentation supports them: E71.511, E71.518, E71.510, E71.540 and others below.
What is the ICD-10 code for rhizomelic chondrodysplasia punctata peroxisomal disorder?
- E71.540 — Rhizomelic chondrodysplasia punctata.
What is the ICD-10 code for x-linked adrenoleukodystrophy peroxisomal disorder?
- E71.529 — X-linked adrenoleukodystrophy, unspecified type.
What is the ICD-10 code for zellweger-like syndrome peroxisomal disorder?
- E71.541 — Zellweger-like syndrome.
Is E71.50 billable?
- Yes. E71.50 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E71.50 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E71.50 as a principal diagnosis.
Is E71.50 a CC or MCC?
- E71.50 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.