ICD-10-CM 2027
ICD-10 code for sphingolipidosis
From the official ICD-10-CM alphabetic index entry “Sphingolipidosis”. Page updated September 29, 2026.
About coding sphingolipidosis
The ICD-10-CM code for sphingolipidosis is E75.3 (Sphingolipidosis, unspecified). The official index lists 1 more specific code, so check the documentation for details such as type, cause, site or severity before settling on E75.3.
Within E75, choose E75.3 (Sphingolipidosis, unspecified) only when documentation doesn't support a more specific option: GM2 gangliosidosis (E75.0), other and unspecified gangliosidosis (E75.1), other sphingolipidosis (E75.2), Neuronal ceroid lipofuscinosis (E75.4), other lipid storage disorders (E75.5) and lipid storage disorder, unspecified (E75.6).
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 sphingolipidosis codes (official index)
- specified NEC E75.29 — Other sphingolipidosis
Common questions about sphingolipidosis ICD-10 codes
What is the ICD-10 code for sphingolipidosis?
- E75.3 — Sphingolipidosis, unspecified. More specific codes apply when the documentation supports them: E75.29.
Is E75.3 billable?
- Yes. E75.3 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E75.3 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E75.3 as a principal diagnosis.
Is E75.3 a CC or MCC?
- No. E75.3 is neither a CC nor an MCC under MS-DRG v44.0.