E76.0 – Mucopolysaccharidosis, type I

ICD-10-CM 2027 diagnosis code · Metabolic disorders

Non-billable header

ICD-10-CM code E76.0

Code: E76.0 (claims format: E760)
Description: Mucopolysaccharidosis, type I
Billable: No – use a more specific code below
Valid for: Dates of service October 1, 2026 – September 30, 2027 (FY2027)
Chapter: 4. Endocrine, nutritional and metabolic diseases (E00-E89)
Block: E70-E88 Metabolic disorders
Parent codes: E76

Billable codes under E76.0

E76.0 is a header code and cannot be used on claims. Choose the most specific code:

Notes inherited from parent codes

Instructional notes at a category or block level apply to every code below it.

Block E70-E88

Excludes1 (not coded here):

androgen insensitivity syndrome (E34.5-)
congenital adrenal hyperplasia (E25.0)
hemolytic anemias attributable to enzyme disorders (D55.-)
Marfan syndrome (Q87.4-)
5-alpha-reductase deficiency (E29.1)

Excludes2 (not included here):

Ehlers-Danlos syndromes (Q79.6-)

Related codes in E76

E76.1 – Mucopolysaccharidosis, type IIBillable
E76.2 – Other mucopolysaccharidosesHeader
E76.3 – Mucopolysaccharidosis, unspecifiedBillable
E76.8 – Other disorders of glucosaminoglycan metabolismBillable
E76.9 – Glucosaminoglycan metabolism disorder, unspecifiedBillable

Frequently asked questions

What is ICD-10 code E76.0?

E76.0 is the ICD-10-CM code for mucopolysaccharidosis, type I, in the block E70-E88 (Metabolic disorders).

Is E76.0 a billable code?

No. E76.0 is a header code with more specific child codes; report one of the billable codes listed above.

What category does E76.0 belong to?

It belongs to category E76 – Disorders of glycosaminoglycan metabolism.

Source: CDC/NCHS ICD-10-CM FY2027 code files, tabular list and index. Reference only – code assignment must follow the official ICD-10-CM guidelines and documentation in the medical record.