ICD-10-CM 2027
ICD-10 code for Mucopolysaccharidosis
From the official ICD-10-CM alphabetic index entry “Mucopolysaccharidosis”. Page updated September 29, 2026.
About coding Mucopolysaccharidosis
The ICD-10-CM code for Mucopolysaccharidosis is E76.3 (Mucopolysaccharidosis, unspecified). The official index lists 23 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on E76.3.
Within E76, choose E76.3 (Mucopolysaccharidosis, unspecified) only when documentation doesn't support a more specific option: Mucopolysaccharidosis, type I (E76.0), Mucopolysaccharidosis, type II (E76.1), other mucopolysaccharidoses (E76.2), other disorders of glucosaminoglycan metabolism (E76.8) and glucosaminoglycan metabolism disorder, unspecified (E76.9).
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.
More specific Mucopolysaccharidosis codes (official index)
- 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
- A E76.210 — Morquio A mucopolysaccharidoses
- B E76.211 — Morquio B mucopolysaccharidoses
- classic E76.210 — Morquio A mucopolysaccharidoses
- Sanfilippo syndrome E76.22 — Sanfilippo mucopolysaccharidoses
- Scheie's syndrome E76.03 — Scheie's syndrome
- specified NEC E76.29 — Other mucopolysaccharidoses
- type
- I
- Hurler's syndrome E76.01 — Hurler's syndrome
- Hurler-Scheie syndrome E76.02 — Hurler-Scheie syndrome
- Scheie's syndrome E76.03 — Scheie's syndrome
- 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
Common questions about Mucopolysaccharidosis ICD-10 codes
What is the ICD-10 code for Mucopolysaccharidosis?
- E76.3 — Mucopolysaccharidosis, unspecified. More specific codes apply when the documentation supports them: E76.29, E76.3, E76.1, E76.01 and others below.
What is the ICD-10 code for beta-gluduronidase deficiency mucopolysaccharidosis?
- E76.29 — Other mucopolysaccharidoses.
What is the ICD-10 code for cardiopathy mucopolysaccharidosis?
- E76.3 — Mucopolysaccharidosis, unspecified.
What is the ICD-10 code for hunter's syndrome mucopolysaccharidosis?
- E76.1 — Mucopolysaccharidosis, type II.
What is the ICD-10 code for hurler's syndrome mucopolysaccharidosis?
- E76.01 — Hurler's syndrome.
What is the ICD-10 code for hurler-scheie syndrome mucopolysaccharidosis?
- E76.02 — Hurler-Scheie syndrome.
What is the ICD-10 code for maroteaux-lamy syndrome mucopolysaccharidosis?
- E76.29 — Other mucopolysaccharidoses.
Is E76.3 billable?
- Yes. E76.3 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E76.3 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E76.3 as a principal diagnosis.
Is E76.3 a CC or MCC?
- E76.3 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.