Aggressive angiomyxoma is a rare benign tumor of the pelvic soft tissue in women of reproductive age. The tumor is locally infiltrative and tends to recur. Herein is presented a case report of aggressive angiomyxoma that was totally excised using a transperineal approach. A 35-year-old woman had an aggressive angiomyxoma of the vulva and pelvis, with swelling of the right labium majus pudendi. Three years previously, she had undergone incomplete excision of the same type of pelviperineal mass via the transabdominal route. After a complete workup, a transperineal minimally invasive approach was used to excise the 20-cm mass filling the right side of the pelvis. Histopathologic findings were consistent with a diagnosis of aggressive angiomyxoma. Although often misdiagnosed as various other benign genital disorders, angiomyxoma usually is manifested as a soft nontender mass. After a thorough examination and full radiologic workup, a small transperineal incision may be sufficient for complete removal of the tumor. Journal of Minimally Invasive Gynecology (2011) 18, 541-544 (C) 2011 AAGL. All rights reserved.