Dieulafoy lesion is an uncommon cause of gastrointestinal (GI) bleeding. Most such lesions are reported in the stomach, though a few have been reported in the distal esophagus. We report a 54-year-old man who presented with upper GI bleeding and had esophageal varices but bled from a Dieulafoy lesion 5 cm above the proximal end of the varices.