Abstract A 14-year-old girl had massive bleeding from a Dieulafoy lesion of the ileum. A preoperative dynamic computed tomography scan detected the point of bleeding. Selective arteriography with embolization using microcoils could not stop the bleeding, but the microcoils were useful as markers of the location of the bleeding point. The position of the microcoils was confirmed by intraoperative fluoroscopy. A partial resection of the ileum that included the lesion was performed. The pathologic finding was Dieulafoy lesion of the ileum. Dieulafoy lesion is a rare condition that usually presents in the stomach. Dieulafoy lesion of the ileum is extraordinarily rare, and to our knowledge, this is only the second case report of an ileal lesion in a child.