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Lymphangioma is a rare benign tumor of lymphatic vessel origin.The tumor often appears in the head and neck region at a young ageand can occasionally be found in the abdomen of adults with proteandegrees of symptoms depending on the tumor size and location. However,lymphangioma of the small intestine is extremely rare with onlya few cases reported in the literature. As the tumor is not well-recognized, many patients with small intestine lymphangioma have beengiven an incorrect preoperative diagnosis. The ideal treatment for thedisease is surgical excision, and the prognosis is comparatively good.In this paper, we report a rare case of ileal lymphangioma with gastrointestinal hemorrhage preoperatively diagnosed using enteroscopyand treated with surgery.