ICD-10-CM 2027
ICD-10 code for von Gierke's disease
From the official ICD-10-CM alphabetic index entry “Disease, diseased › von Gierke's (glycogenosis I)”. Page updated September 29, 2026.
About coding von Gierke's disease
The ICD-10-CM code for von Gierke's disease is E74.01 (von Gierke disease).
Within E74.0 (glycogen storage disease), E74.01 is specifically for von Gierke disease. Related codes cover unspecified (E74.00), Pompe disease (E74.02), Cori disease (E74.03), McArdle disease (E74.04), Lysosome-associated membrane protein 2 [LAMP2] deficiency (E74.05) and other glycogen storage disease (E74.09).
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 von Gierke's disease ICD-10 codes
What is the ICD-10 code for von Gierke's disease?
- E74.01 — von Gierke disease.
Is E74.01 billable?
- Yes. E74.01 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E74.01 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E74.01 as a principal diagnosis.
Is E74.01 a CC or MCC?
- E74.01 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.