The experience of a specialized management team using urgent endoscopy in the management of acute gastrointestinal bleeding from Dieulafoy's disease is presented. Dieulafoy's disease was found in 19 of 1124 consecutive patients with upper gastrointestinal bleeding. Most patients with Dieulafoy's disease were elderly men with severe acute upper gastrointestinal hemorrhage. Endoscopic diagnosis was possible in all patients, but required multiple endoscopies in 37%. The lesions were in the proximal stomach (79%) and duodenal bulb (21%). Endoscopic therapy included epinephrine injection, then heater probe coagulation in 17 patients, bipolar electrocoagulation in 1, and Nd:YAG laser photocoagulation in 1. Endoscopic therapy was successful in 18 patients (95%); one patient had successful surgery after endoscopic therapy failed. There were no deaths due to bleeding and no endoscopic complications. Dieulafoy's disease is an unusual cause of acute gastrointestinal bleeding. Endoscopic diagnosis is sometimes difficult, but primary endoscopic therapy is safe, successful, and should be attempted.