Background: Aggressive angiomyxoma (AA) is a rare tumor that usually appears in the female pelvic and perineal regions. It commonly has infiltrative behavior and high local recurrence risk. We report an unusual presentation of AA, originating in a female patient's bladder. Case Report: A 43-year-old female patient presented with recurrent urinary tract infection for 6 months; ultrasonography showed a bladder tumor that was diagnosed as AA by immunohistochemistry and treated with complete transurethral resection. Conclusion: Transurethral resection can be an effective approach for the treatment of AA.