Nine female patients with the rare congenital arteriovenous malformation of the pelvis were treated at New York University Medical Center in the past 8 years. The patients varied significantly in the anatomic location of the arteriovenous malformation, clinical presentation, and natural course of disease. A gynecologic perspective on the management of this condition is presented, which ranges from a conservative approach with preservation of childbearing potential to extensive pelvic surgery. The important role of modern interventional radiology technology and the multidisciplinary approach are stressed. The unpredictable course of disease after any intervention should be emphasized in planning the management of pelvic arteriovenous malformation.