ICD-10-CM 2027
ICD-10 code for glycogen storage disease
From the official ICD-10-CM alphabetic index entry “Disease, diseased › glycogen storage”. Page updated September 29, 2026.
About coding glycogen storage disease
The ICD-10-CM code for glycogen storage disease is E74.00 (Glycogen storage disease, unspecified). The official index lists 26 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on E74.00.
Within E74.0, choose E74.00 (unspecified) only when documentation doesn't support a more specific option: von Gierke disease (E74.01), 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.
More specific glycogen storage disease codes (official index)
- Andersen's E74.09 — Other glycogen storage disease
- Cori's E74.03 — Cori disease
- Forbes' E74.03 — Cori disease
- generalized E74.00 — Glycogen storage disease, unspecified
- glucose-6-phosphatase deficiency E74.01 — von Gierke disease
- heart E74.02 — Pompe disease
- hepatorenal E74.09 — Other glycogen storage disease
- Hers' E74.09 — Other glycogen storage disease
- liver and kidney E74.09 — Other glycogen storage disease
- lysosomal E74.02 — Pompe disease
- with acid maltase deficiency E74.02 — Pompe disease
- without acid maltase deficiency E74.05 — Lysosome-associated membrane protein 2 [LAMP2] deficiency
- McArdle's E74.04 — McArdle disease
- muscle phosphofructokinase E74.09 — Other glycogen storage disease
- myocardium E74.02 — Pompe disease
- Pompe's E74.02 — Pompe disease
- Tauri's E74.09 — Other glycogen storage disease
- type 0 E74.09 — Other glycogen storage disease
- type I E74.01 — von Gierke disease
- type II E74.02 — Pompe disease
- type IIB E74.05 — Lysosome-associated membrane protein 2 [LAMP2] deficiency
- type III E74.03 — Cori disease
- type IV E74.09 — Other glycogen storage disease
- type V E74.04 — McArdle disease
- type VI-XI E74.09 — Other glycogen storage disease
- Von Gierke's E74.01 — von Gierke disease
Common questions about glycogen storage disease ICD-10 codes
What is the ICD-10 code for glycogen storage disease?
- E74.00 — Glycogen storage disease, unspecified. More specific codes apply when the documentation supports them: E74.09, E74.03, E74.03, E74.00 and others below.
What is the ICD-10 code for andersen's glycogen storage disease?
- E74.09 — Other glycogen storage disease.
What is the ICD-10 code for cori's glycogen storage disease?
- E74.03 — Cori disease.
What is the ICD-10 code for forbes' glycogen storage disease?
- E74.03 — Cori disease.
What is the ICD-10 code for generalized glycogen storage disease?
- E74.00 — Glycogen storage disease, unspecified.
What is the ICD-10 code for glucose-6-phosphatase deficiency glycogen storage disease?
- E74.01 — von Gierke disease.
What is the ICD-10 code for heart glycogen storage disease?
- E74.02 — Pompe disease.
Is E74.00 billable?
- Yes. E74.00 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E74.00 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E74.00 as a principal diagnosis.
Is E74.00 a CC or MCC?
- E74.00 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.