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