Aggressive angiomyxoma (AAM) is a rare locally aggressive tumor affecting the pelvis and perineum of young females. Histopathologically, it is characterized by fibreoblasts in a myxoid background with vascular proliferation, scanty mitotic figure and no real capsule. AAM needs to be considered in the differential diagnosis of vulval mass in a reproductive age woman. We describe the first Ethiopian case of huge, recurrent, AAM of the pelvis and perineum in a 35 year old para II lady and stress that early diagnosis offers a better option for treatment by wide local excision and low recurrence rate.