ICD-10-CM 2027
ICD-10 code for Maple-syrup-urine disease
From the official ICD-10-CM alphabetic index entry “Maple-syrup-urine disease”. Page updated September 29, 2026.
About coding Maple-syrup-urine disease
The ICD-10-CM code for Maple-syrup-urine disease is E71.0 (Maple-syrup-urine disease).
Within E71 (disorders of branched-chain amino-acid metabolism and fatty-acid metabolism), E71.0 is specifically for Maple-syrup-urine disease. Related codes cover other disorders of branched-chain amino-acid metabolism (E71.1), disorder of branched-chain amino-acid metabolism, unspecified (E71.2), disorders of fatty-acid metabolism (E71.3), disorders of carnitine metabolism (E71.4) and peroxisomal disorders (E71.5).
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.
Common questions about Maple-syrup-urine disease ICD-10 codes
What is the ICD-10 code for Maple-syrup-urine disease?
- E71.0 — Maple-syrup-urine disease.
Is E71.0 billable?
- Yes. E71.0 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E71.0 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E71.0 as a principal diagnosis.
Is E71.0 a CC or MCC?
- E71.0 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.