ICD-10-CM 2027
ICD-10 code for Isovaleric acidemia
From the official ICD-10-CM alphabetic index entry “Acidemia › isovaleric”. Page updated September 29, 2026.
About coding Isovaleric acidemia
The ICD-10-CM code for Isovaleric acidemia is E71.110 (Isovaleric acidemia).
Within E71.11 (branched-chain organic acidurias), E71.110 is specifically for Isovaleric acidemia. Related codes cover 3-methylglutaconic aciduria (E71.111) and other branched-chain organic acidurias (E71.118).
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 Isovaleric acidemia ICD-10 codes
What is the ICD-10 code for Isovaleric acidemia?
- E71.110 — Isovaleric acidemia.
Is E71.110 billable?
- Yes. E71.110 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E71.110 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E71.110 as a principal diagnosis.
Is E71.110 a CC or MCC?
- E71.110 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.