ICD-10 Code E71.120 – Methylmalonic acidemia (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Methylmalonic acidemia is E71.120.
2026 ICD-10-CM Diagnosis Code E71.120 – Methylmalonic acidemia

What it is

Methylmalonic acidemia is an inherited metabolic disorder in which the body cannot properly process certain fats and proteins. It leads to buildup of methylmalonic acid and related metabolic products.

Clinical signs

Clinical features vary; refer to documentation. Common findings may include recurrent vomiting, poor feeding, lethargy, developmental delay, and episodes of metabolic decompensation.

When to use this code

Use E71.120 when the record clearly documents methylmalonic acidemia as the confirmed diagnosis. It may also apply when the condition is being followed long term as a known inherited disorder. Code the documented subtype or associated disorder separately if the record supports it.

Do not use for

Do not use this code for isolated elevated methylmalonic acid without a diagnosis of methylmalonic acidemia. Check documentation if the note only states suspected metabolic disorder or abnormal screening results.

Coding tip

Confirm the provider’s final diagnosis and avoid coding from laboratory abnormalities alone.

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