Aggressive angiomyxoma is a rare neoplasm which predominantly involves the female pelvis and perineum. Forty-four cases have been reported in the world literature, including 10 cases in men. To the best of our knowledge, the first case of recurrent aggressive angiomyxoma of the urinary bladder is presented here. Operative management, radiologic features and pathological findings are discussed. Copyright 2000 S. Karger AG, Basel