Vascular lesions of the colon are being recognized with increasing frequency as a cause of lower intestinal hemorrhage in the elderly [1–3]. Colonic arteriovenous malformation (AVM) is also known as vascular ectasia or angiodysplasia, and it is thought to be caused by degenerative changes [1, 4]. Unlike small vascular abnormalities such as vascular ectasia or angiodysplasia, AVMs have several distinguishing characteristics based on the analyses of various reported cases [4–6]: they are not restricted to the elderly, are usually solitary, can be identified endoscopically, are not confined to the right colon, and are larger. We report a case of segmental AVM in a patient with familial adenomatous polyposis (FAP) that presented as a chronic limited colitis mimicking inflammatory bowel disease. While initial biopsies were suggestive of an ischemic etiology, the final diagnosis was made on surgical resection.