ICD-10 Code E71.511 – Neonatal adrenoleukodystrophy (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Neonatal adrenoleukodystrophy is E71.511.
2026 ICD-10-CM Diagnosis Code E71.511 – Neonatal adrenoleukodystrophy

What it is

E71.511 identifies neonatal adrenoleukodystrophy, a rare inherited disorder affecting very-long-chain fatty acid metabolism. It is used when documentation specifically names this neonatal form.

Clinical signs

Clinical features vary; refer to documentation. Standard references describe early neurologic and developmental abnormalities, and possible adrenal involvement, in affected newborns.

When to use this code

Use this code when the record clearly documents neonatal adrenoleukodystrophy as the diagnosis. It may appear in newborn or infant assessments, specialist consultations, or problem lists tied to confirmed disease. If the chart only says “adrenoleukodystrophy” without the neonatal form, check documentation.

Do not use for

Do not use it for other leukodystrophies, nonspecific peroxisomal disorders, or suspected disease without confirmation. Check documentation if the term is abbreviated or the neonatal subtype is not stated.

Coding tip

Code the most specific documented leukodystrophy subtype; if the neonatal form is not explicit, verify the diagnosis before assigning E71.511.

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