A case of sudden death is reported in a 13-year-old boy due to an acute myocardial infarction and arrhythmias following exertion. On autopsy, he was found to have an anomalous origin of the left coronary artery, an acute angle of takeoff of the left coronary artery, and diffuse hypoplasia of both the left and right coronary arteries. Both arteries had markedly small lumens throughout their course. This report provides the first description of this combination of congenital coronary artery abnormalities resulting in sudden death.