Congenital arteriovenous malformation of the extracranial region is rare. The case presented here is a woman aged 29. She gave a history of swelling of the scalp at the left temporat region and noticed a rhythmic pulsating noise in the left ear of five mon ths duration, especially when she placed that side of her head against a pillow. Physical examination revealed a pulsating compressible tumor mass measuring 10 cmx8cm in the left temporal area of the head. There was a bruit synchronous with the pulse and an associated thrill over the mass. A left external carotid angiogram demonstrated that the deep temporat branches of the marked dilated internal maxillary artery as well as the branch of the superficial temporal artery fed the arteriovenous malformation at the extracranial region. Venous drainage to the superficial temporal vein and the maxillary vein were present. Multiple arteriovenous fistulas and persistent pooling of contrast medium were noted. At operation. At operation, the left external carotid artery was ligated and the malformation was excised. The postoperative course was entirely uncomplicated. The bruit disappeared and she was discharged on the 12th postoperative day. Histologically, the arteriovenous malformation was demonstrated. We reviewed the literatures and discussed the etiology, clinical and roentgraphic findings of the congenital arteriovenous malformation at the extracranial region.