Varices that occur at sites other than the esophagogastric area are termed ectopic varices. An ileal varix is a very rare cause of lower gastrointestinal bleeding. Although ileal varices are generally associated with prior intra-abdominal surgery and adhesions, an arteriovenous malformation (AVM) in the ileocecal area can cause ileal varices and bleeding in patients with portal hypertension who have not received previous intra-abdominal surgery, which is due to an intestinal or colonic AVM dilating the collateral veins and further aggravating portal hypertension. Surgical treatment should be considered in patients with massive ectopic variceal bleeding. We report a case of massive ileocecal variceal bleeding associated with an AVM that occurred in a patient with alcoholic liver cirrhosis. (Gut and Liver 2008;2:54-59)