We report an autopsy case of a 19-year-old male who died suddenly. Death happened while he was sleeping after drinking alcoholic beverages. His heart revealed concentric hypertrophy of the left ventricle without asymmetric septal hypertrophy or mitral regurgitation. Upon microscopic examination, the epicardial atrioventricular (AV) node artery revealed stenosis with intimal thickening before it entered the ventricular septum and acute ischemic change was observed beneath the conduction system in the upper ventricular septum. This finding suggests that death occurred after some preceding localized ischemic event within the ventricular septum. We therefore consider that the cause of death was fatal arrhythmia due to dysplastic change to the AV node artery.