ICD-10 Code E71.542 – Other group 3 peroxisomal disorders (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Other group 3 peroxisomal disorders is E71.542.
2026 ICD-10-CM Diagnosis Code E71.542 – Other group 3 peroxisomal disorders

What it is

E71.542 identifies other group 3 peroxisomal disorders, a category of inherited metabolic conditions affecting peroxisome function. These disorders can disrupt normal breakdown of certain fats and other cellular processes.

Clinical signs

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

When to use this code

Use this code when the provider documents a specific group 3 peroxisomal disorder that does not fit a more precise ICD-10-CM option. It is appropriate for confirmed diagnoses in the chart, genetic workup, or specialist assessment. Check documentation if the record only says “peroxisomal disorder” without further detail.

Do not use for

Do not use this code for other peroxisomal disorders with a more specific diagnosis, or for suspected disease without confirmation. Do not assign it when the documented condition belongs to a different metabolic or genetic category.

Coding tip

Code to the highest documented specificity and verify the exact disorder name before selecting E71.542.

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