ICD-10-CM 2027 diagnosis code

E76.0Mucopolysaccharidosis, type I

Not billable — use a more specific code

E76.0 is a header code, so it can't be reported by itself. Choose one of the 3 more specific codes beneath it.

Code unchanged since ICD-10-CM took effect on October 1, 2015. Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data

About E76.0

E76.0 is the ICD-10-CM diagnosis code for Mucopolysaccharidosis, type I. It belongs to category E76 (disorders of glycosaminoglycan metabolism), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is a header code, so it can't be reported by itself; one of its 3 subcodes must be used instead.

Within E76 (disorders of glycosaminoglycan metabolism), E76.0 is specifically for Mucopolysaccharidosis, type I. Related codes cover Mucopolysaccharidosis, type II (E76.1), other mucopolysaccharidoses (E76.2), Mucopolysaccharidosis, unspecified (E76.3), other disorders of glucosaminoglycan metabolism (E76.8) and glucosaminoglycan metabolism disorder, unspecified (E76.9).

To report this condition, pick the subcode that matches the documentation: E76.01 (syndrome), E76.02 (syndrome) and E76.03 (syndrome).

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Specific codes under E76.0

Notes that apply from higher levels

Instructions written at a parent level also apply to E76.0.

Broader instructions also apply from E70-E88 Metabolic disorders and Chapter 4: Endocrine, nutritional and metabolic diseases.

Codes whose notes reference E76.0

E74Excludes1
Q77Excludes1

Code history

2016
2017
2018
2019
2020
2021
2022
2023
2024
2025
2026
2027

FY2016 (effective 10/1/2015): Added (first year of ICD-10-CM)
No changes since FY2016.

Common questions about E76.0

Is E76.0 billable?: No. E76.0 is a header code. Report one of its more specific subcodes instead.