E71.0 – Maple-syrup-urine disease

ICD-10-CM 2027 diagnosis code · Metabolic disorders

Billable code

ICD-10-CM code E71.0

Code: E71.0 (claims format: E710)
Description: Maple-syrup-urine disease
Billable: Yes – valid for HIPAA-covered transactions
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: E71

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-)

Index terms for E71.0

Entries in the ICD-10-CM alphabetic index that lead to this code:

Related codes in E71

Frequently asked questions

What is ICD-10 code E71.0?

E71.0 is the ICD-10-CM code for maple-syrup-urine disease, in the block E70-E88 (Metabolic disorders).

Is E71.0 a billable code?

Yes. E71.0 is a billable/specific code that can be used to indicate a diagnosis for reimbursement purposes.

What category does E71.0 belong to?

It belongs to category E71 – Disorders of branched-chain amino-acid metabolism and fatty-acid 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.