Dieulafoy lesion is a rare cause of upper gastrointestinal bleeding. We present a case report of sudden and unexpected death in a previously healthy male where autopsy revealed a classic Dieulafoy lesion with a submucosal artery protruding through a small mucosal defect in the esophagus, thereby exposing it to stress and consequent rupture. Advances in endoscopic techniques have drastically reduced mortality from Dieulafoy lesion. Nevertheless, it is an important diagnosis to be kept in mind when autopsy reveals an upper gastrointestinal bleeding.