ICD-10-CM 2027
ICD-10 code for Zellweger syndrome syndrome
From the official ICD-10-CM alphabetic index entry “Syndrome › Zellweger syndrome”. Page updated September 29, 2026.
About coding Zellweger syndrome syndrome
The ICD-10-CM code for Zellweger syndrome syndrome is E71.510 (Zellweger syndrome).
Within E71.51 (disorders of peroxisome biogenesis), E71.510 is specifically for Zellweger syndrome. Related codes cover neonatal adrenoleukodystrophy (E71.511) and other disorders of peroxisome biogenesis (E71.518).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Zellweger syndrome syndrome ICD-10 codes
What is the ICD-10 code for Zellweger syndrome syndrome?
- E71.510 — Zellweger syndrome.
Is E71.510 billable?
- Yes. E71.510 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E71.510 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E71.510 as a principal diagnosis.
Is E71.510 a CC or MCC?
- E71.510 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.