ICD-10 Code for Mucopolysaccharidosis
The ICD-10-CM code for mucopolysaccharidosis is E76.3 — Mucopolysaccharidosis, unspecified. This is the code the Alphabetic Index gives for mucopolysaccharidosis with no further qualifier, valid for FY2027 (October 1, 2026 – September 30, 2027).
E76.3 is billable but unspecified. If the documentation names a type, site or cause, one of the more specific codes below is likely expected.
Which mucopolysaccharidosis code should you use?
The Alphabetic Index lists 11 codes under Mucopolysaccharidosis. Which one applies depends on the qualifier documented in the record — the type, site, cause, or associated condition. Find the qualifier below, then verify the code in the Tabular List before using it.
By type
| beta-gluduronidase deficiency | E76.29 | Other mucopolysaccharidoses | |
| cardiopathy | E76.3 | Mucopolysaccharidosis, unspecified | |
| Hunter's syndrome | E76.1 | Mucopolysaccharidosis, type II | |
| Hurler's syndrome | E76.01 | Hurler's syndrome | |
| Hurler-Scheie syndrome | E76.02 | Hurler-Scheie syndrome | |
| Maroteaux-Lamy syndrome | E76.29 | Other mucopolysaccharidoses | |
| Morquio syndrome | E76.219 | Morquio mucopolysaccharidoses, unspecified | |
| Sanfilippo syndrome | E76.22 | Sanfilippo mucopolysaccharidoses | |
| Scheie's syndrome | E76.03 | Scheie's syndrome | |
| specified NEC | E76.29 | Other mucopolysaccharidoses |
Mucopolysaccharidosis Morquio syndrome
Mucopolysaccharidosis type
| II | E76.1 | Mucopolysaccharidosis, type II | |
| III | E76.22 | Sanfilippo mucopolysaccharidoses | |
| IV | E76.219 | Morquio mucopolysaccharidoses, unspecified | |
| IVA | E76.210 | Morquio A mucopolysaccharidoses | |
| IVB | E76.211 | Morquio B mucopolysaccharidoses | |
| VI | E76.29 | Other mucopolysaccharidoses | |
| VII | E76.29 | Other mucopolysaccharidoses | |
| I › Hurler's syndrome | E76.01 | Hurler's syndrome | |
| I › Hurler-Scheie syndrome | E76.02 | Hurler-Scheie syndrome | |
| I › Scheie's syndrome | E76.03 | Scheie's syndrome |
Index terms and codes are reproduced from the official ICD-10-CM FY2027 release. The Alphabetic Index is a finding aid — a code must always be verified in the Tabular List before it is reported. Data sources