Objectives　To explore the clinical diagnosis and treatment of Dieulafoy disease in children. Method　The clinical features, endoscopic features and treatment of Dieulafoy disease in a child were reviewed. Results　The 2-year-5month old girl was admitted due to hematemesis for 7 hours. She was diagnosed of Dieulafoy disease by the typical endoscopic appearance. Gastroscopy showed that the lesion was located in gastric angle which was the predilection position of Dieulafoy disease. The small red blood vessels in the central part of the erosion area was exposed on the mucosal surface. The high frequency electrocoagulation under gastroscope was performed and effect was definite. Conclusion　Dieulafoy disease is rare in children and lacks obvious clinical features. Endoscopic treatment has definite effect with little trauma and is the first choice of treatment.