A localized arteriovenous malformation of the bladder is extremely rare. To our knowledge, arteriovenous malformation of the bladder has been reported in limited number of cases in the published literature. When arteriovenous malformation is suspected, an angiogram and pelvic computed tomography or magnetic resonance imaging is essential to delineate the extent of the disease and to plan appropriate therapy. Common symptoms include pain, gross hematuria and acute urinary retention. No well-established guidelines exist concerning their management. We report a case of an arteriovenous malformation of the urinary bladder that was successfully treated by transurethral resection.