A patient with a large congenital pelvic arteriovenous malformation presenting with irradiating pain to the leg, most likely due to sciatic nerve compression, is described. Congenital pelvic arteriovenous malformation are rare lesions, especially in males. Diagnosis was established by arteriography and contrast-enhanced computed tomography scan. Surgical resection of the AVM relieved our patient of all symptoms. However, endovascular therapy, either as primary treatment or followed by conventional surgery is the treatment of choice. Pelvic arteriovenous malformations should be considered in the differential diagnosis of unexplained sciatica.