The glycogen storage diseases are a rare form of inherited metabolic disease affecting intracellular glycogen metabolism, and several studies suggest glycogen storage disease (GSD) III predisposes patients to dyslipidemia and endothelial dysfunction. The presence of premature atherosclerotic heart disease in patients with GSD III has not been reported in the literature. We report a case of a 24- year old patient with GSD III admitted with ventricular fibrillation cardiac arrest in the setting of anterior wall myocardial infarction. Further studies are warranted on the prevalence of atherosclerotic heart disease, and potential screening and preventative strategies, in this population of patients potentially at-risk for early cardiac events.