Aggressive angiomyxoma is a locally mesenchymal, benign, and rare neoplasm. The vulva, perineum, and pelvis are the most common sites of involvement. The preoperative diagnosis is postulated by CT, sonography, MR image and angiography. The immunohistochemical study reveals the definitive diagnosis. The therapy is only surgical, and because of its tendency to recur locally, the excision has to be as complete as possible.