Aggressive angiomyxoma (AAM) is a rare and nonmetastasizing soft-tissue tumor predominantly found in the female pelvis and perineum. It has a high risk of local recurrence. We report the unusual case of a 15-year-old boy with an AAM presenting as a slowly enlarging scrotal mass. The patient had had chronic renal failure since 1997 and had needed hemodialysis for the previous 11 months. He presented with a 12-month history of a nontender soft mass in the right scrotum. Ultrasound examination revealed a solid mass in the scrotum. After surgical resection, pathological analysis disclosed spindle-shaped neoplastic cells widely separated by a myxoid stroma rich in collagen fibers and prominent irregularly shaped blood vessels; the histological examination confirmed an AAM.