Dieulafoy's disease, a vascular anomaly mainly in the upper stomach, is a rare but potentially life-threatening cause of upper gastrointestinal bleeding. Pathogenesis is still controversial, but the most accepted theory is that a persistent caliber vessel in the submucosa is exposed by a small mucosal erosion leading to massive bleeding. The bleeding site is usually within 6 cm of the esophagogastric junction in the cardia or fundus of the stomach. The treatment of choice is therapeutic endoscopy or surgery. The age of patients reported is mainly between 50 and 70 years, and patients of pediatric age are extremely rare. We are reporting a 5-year-old male patient who had Dieulafoy's disease which was diagnosed by emergency upper gastrointestinal endoscopy. Endoscopic finding was a nodular lesion with an adherent clot on the lessor curvature of the stomach 2 cm below the esophagogastric junction. Epinephrine and Beriplast(R) was injected in the lesion. On the second day after endoscopic sclerotherapy, the patient had recurred massive hematemesis and accompanying shock. So we performed gastrotomy and ligation. After the operation, he showed an improved general condition and was discharged at the 12th hospital day.