ICD-10 Code E75.29 – Other sphingolipidosis (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Other sphingolipidosis is E75.29.
2026 ICD-10-CM Diagnosis Code E75.29 – Other sphingolipidosis

What it is

Other sphingolipidosis is a group code for inherited disorders of sphingolipid breakdown that do not fit a more specific ICD-10 category. These conditions cause abnormal lipid accumulation in cells and tissues.

Clinical signs

Clinical features vary; refer to documentation. Common findings in sphingolipidoses may include neurologic decline, organ enlargement, skeletal changes, or developmental delay, depending on the specific disorder.

When to use this code

Use E75.29 when the record documents a sphingolipidosis but does not name a more specific type. It also fits when the clinician describes an inherited sphingolipid storage disorder and no alternate code is supported. Check documentation if the exact diagnosis is unclear.

Do not use for

Do not use this code for a clearly identified sphingolipidosis with its own specific ICD-10 code. Do not assign it for nonspecific lipid disorders or for suspected disease without confirmation.

Coding tip

Verify the exact lysosomal storage diagnosis in the chart before coding, and default to the most specific code available.

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