Dieulafoy disease is an unusual cause of gastrointestinal (GI) hemorrhage that can be fatal. It arises from an abnormally large eroded submucosal artery commonly located in the proximal stomach. Since this disease first described in 1884, it has been rarely discussed in radiology literature. We describe a case of an elderly patient with multiple medical problems presented with upper and lower GI hemorrhage. Repeated upper GI endoscopies revealed a large fundal clot but otherwise were unremarkable. Angiography showed anomalous short gastric artery originating normally from the splenic artery. The latter was embolized with microcoils and Gelfoam®. Following embolization, the frequency and amount of bleeding decreased although did not totally stop and the patient underwent elective partial gastrectomy. The diagnosis was made histologically. Therefore, Dieulafoy disease can represent a diagnostic and therapeutic challenge. It should be suspected if a patient with GI hemorrhage is found to have extravasation from a dilated tortuous artery that appears normal otherwise with no associated large draining veins. Transcatheter selective arterial embolization has a reported role in the literature as a less invasive therapeutic option compared to surgery.