ICD-10 Code E71.518 – Other disorders of peroxisome biogenesis (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Other disorders of peroxisome biogenesis is E71.518.
2026 ICD-10-CM Diagnosis Code E71.518 – Other disorders of peroxisome biogenesis

What it is

E71.518 identifies a peroxisome biogenesis disorder not otherwise specified elsewhere. Use it when documentation shows abnormal peroxisome formation or function, but the record does not support a more specific peroxisomal diagnosis.

Clinical signs

Clinical features vary; refer to documentation. Common references describe developmental delay, hypotonia, seizures, vision or hearing problems, and liver or neurologic involvement in peroxisomal disorders.

When to use this code

Use this code when the clinician documents a peroxisome biogenesis disorder and does not name a more specific subtype. It may also fit cases described as “other” or unspecified peroxisomal biogenesis abnormalities. Check documentation if genetic or biochemical details are incomplete.

Do not use for

Do not use it for a clearly identified peroxisomal syndrome or another metabolic disorder with its own code. Do not assume this code from nonspecific neurologic or hepatic findings alone.

Coding tip

Code from the provider’s exact wording, and confirm whether a more specific peroxisomal diagnosis is documented before assigning E71.518.

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