A patient with recurrent gastrointestinal bleeding from the distal jejunal lymphangioma was diagnosed preoperatively. The diagnosis was made by using double-balloon enteroscopy with biopsy. Endoscopic examination showed a yellowish-whitish surface with red and white specks indicate of a polypoid tumor. The patient received the laparoscopic assisted surgery to remove the lesion and no recurrent symptom was noted after two years followup. Lymphangioma of the duodenum and colon may be successfully diagnosed preoperatively by routine endoscopy. Nonetheless, such a diagnosis is hard to make when the lesion is in the distal small intestine. Double-balloon enteroscopy is a useful diagnostic tool, and rarely accurately diagnosed preoperatively for lymphangioma of the small intestine in a patient with otherwise unexplained overt gastrointestinal bleeding.