A case of aggressive angiomyxoma occurring in the scrotum is reported. A 57-year-old Japanese man presented with a 2-year history of non-tender soft mass in the scrotum. Computed tomography and magnetic resonance imaging revealed a well-defined mass 8 cm in diameter extending from the medial aspect of the left ischium. Angiography revealed faint vascularity fed by the left internal pudendal artery. Excision was performed by a scrotal approach, and histological examination confirmed an aggressive angiomyxoma. The patient showed no recurrence at 3 months follow-up.