ICD-10 Code E71.541 – Zellweger-like syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.541 – Zellweger-like syndrome
What it is
E71.541 identifies Zellweger-like syndrome, a peroxisomal disorder that resembles Zellweger syndrome. It is used when documentation specifically names this phenotype or a closely related Zellweger-like presentation.
Clinical signs
Clinical features vary; refer to documentation. Commonly described findings in peroxisomal disorders include hypotonia, developmental delay, feeding difficulty, and abnormalities affecting the liver, brain, or eyes.
When to use this code
Use this code when the provider documents Zellweger-like syndrome as the diagnosis, including confirmed or suspected cases recorded in the chart. It may also fit when the record describes a Zellweger-like phenotype without a more specific named peroxisomal disorder. Check documentation if the note instead names classic Zellweger syndrome or another metabolic condition.
Do not use for
Do not use this code for nonspecific developmental delay, hypotonia, or liver disease without documentation of Zellweger-like syndrome. If the chart names a different peroxisomal disorder, code that condition instead.
Coding tip
Code from the exact diagnostic wording in the provider note and confirm whether “Zellweger-like” is explicitly stated.