ICD-10-CM 2027
ICD-10 code for Niemann-Pick disease or syndrome
From the official ICD-10-CM alphabetic index entry “Niemann-Pick disease or syndrome”. Page updated September 29, 2026.
About coding Niemann-Pick disease or syndrome
The ICD-10-CM code for Niemann-Pick disease or syndrome is E75.249 (Niemann-Pick disease, unspecified). The official index lists 6 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on E75.249.
Within E75.24, choose E75.249 (unspecified) only when documentation doesn't support a more specific option: type A (E75.240), type B (E75.241), type C (E75.242), type D (E75.243), type A/B (E75.244) and other Niemann-Pick disease (E75.248).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
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.
More specific Niemann-Pick disease or syndrome codes (official index)
Common questions about Niemann-Pick disease or syndrome ICD-10 codes
What is the ICD-10 code for Niemann-Pick disease or syndrome?
- E75.249 — Niemann-Pick disease, unspecified. More specific codes apply when the documentation supports them: E75.248, E75.240, E75.244, E75.241 and others below.
Is E75.249 billable?
- Yes. E75.249 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E75.249 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E75.249 as a principal diagnosis.
Is E75.249 a CC or MCC?
- No. E75.249 is neither a CC nor an MCC under MS-DRG v44.0.