ICD-10 Code E71.54 – Other peroxisomal disorders (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Other peroxisomal disorders is E71.54.
2026 ICD-10-CM Diagnosis Code E71.54 – Other peroxisomal disorders

What it is

E71.54 identifies peroxisomal disorders that do not fit a more specific ICD-10-CM category. These are rare inherited conditions affecting peroxisome function, which can disrupt normal metabolism.

Clinical signs

Clinical features vary; refer to documentation. Commonly reported findings in peroxisomal disorders include developmental delay, neurologic impairment, vision or hearing problems, and liver involvement.

When to use this code

Use E71.54 when the provider documents an unspecified or other peroxisomal disorder and no more specific peroxisomal diagnosis is stated. It is appropriate when the record confirms a peroxisomal condition but does not name a distinct subtype. Check documentation if genetic testing or specialist notes identify a specific disorder.

Do not use for

Do not use this code for a clearly documented specific peroxisomal disorder that has its own ICD-10-CM code. Do not assign it when the chart only suggests a metabolic disorder without confirmation.

Coding tip

Code the most specific peroxisomal diagnosis available; if the subtype is unclear, use E71.54 only when the provider explicitly supports “other” peroxisomal disorder.

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