ICD-10 Code E71.111 – 3-methylglutaconic aciduria (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.111 – 3-methylglutaconic aciduria
What it is
E71.111 identifies 3-methylglutaconic aciduria, a rare inherited metabolic disorder in which the body cannot process certain organic acids normally. It is used when this specific diagnosis is documented.
Clinical signs
Clinical features vary; refer to documentation. Common references describe developmental delay, neurologic abnormalities, muscle weakness, and elevated 3-methylglutaconic acid on metabolic testing.
When to use this code
Use this code when the provider documents 3-methylglutaconic aciduria, including confirmed metabolic or genetic diagnoses. It may also apply when the condition is the reason for evaluation or ongoing management. Do not assume it from nonspecific neurologic or metabolic symptoms alone.
Do not use for
Do not use this code for unrelated organic acidemias or for abnormal screening results without a documented diagnosis. Check documentation if the chart only notes suspected metabolic disease.
Coding tip
Verify the exact disorder name in the assessment or problem list before assigning E71.111.