Arteriovenous (AV) malformation or angiodysplasia of the gastrointestinal tract is a very rare cause of bleeding in children. These lesions are congenital anomalies and thought to be of hamartomatous origin. We report on a 4-year-old child with an AV malformation of the distal ileum treated by local resection. A brief review of the relevant literature is included. The importance of radionuclide blood pool scanning in the evaluation of these patients is emphasized.