ICD-10 Code E71.510 – Zellweger syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.510 – Zellweger syndrome
What it is
Zellweger syndrome is a rare inherited peroxisomal disorder that affects multiple organs and development. Use this code when documentation identifies the specific Zellweger syndrome diagnosis.
Clinical signs
Clinical features vary; refer to documentation. Commonly described findings include severe hypotonia, feeding difficulty, developmental delay, craniofacial abnormalities, and liver or neurologic involvement.
When to use this code
Use E71.510 when the provider documents Zellweger syndrome by name, including confirmed or suspected cases supported in the record. It may also be appropriate when the chart specifies a Zellweger spectrum disorder and the clinician equates it to this diagnosis. Check documentation if the record only mentions peroxisomal disease without naming Zellweger syndrome.
Do not use for
Do not use this code for other peroxisomal disorders, nonspecific metabolic disease, or a family history only. If the diagnosis is uncertain or not stated, check documentation.
Coding tip
Verify the exact syndrome name in the assessment or discharge diagnosis before assigning E71.510.