ICD-10 Code E74.01 – von Gierke disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E74.01 – von Gierke disease
What it is
E74.01 identifies von Gierke disease, also called glycogen storage disease type I. It is an inherited disorder that affects the body’s ability to break down and release stored glycogen normally.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include enlarged liver, poor growth, and episodes related to low blood sugar. You should code only when the diagnosis is clearly documented.
When to use this code
Use E74.01 when the provider documents von Gierke disease, glycogen storage disease type I, or a confirmed equivalent term. It may appear in pediatric metabolic records, genetic evaluations, or problem lists. If the note only says hypoglycemia or hepatomegaly, do not assume this diagnosis.
Do not use for
Do not use this code for unspecified glycogen storage disease or other glycogen storage disorders. Check documentation if the record names a different metabolic condition or only describes symptoms without a confirmed diagnosis.
Coding tip
Match the exact documented metabolic disorder; when the type is not stated, query the provider rather than defaulting to E74.01.