Aggressive angiomyxoma is a rare benign mesenchymal neoplasm, found mainly in the female pelvis and perineum. Approximately 160 cases have been reported in the literature to date. The tumor is usually misdiagnosed at presentation. Surgical and margin-free excision is considered the treatment of choice. Other treatment options include use of hormone antagonists, such as tamoxifen, or GnRH analogs, and selective angiographic embolism. High recurrence rate of the tumor, up to 70% within a 2-year period, makes long-term imaging follow-up of the patient necessary.