A case of arteriovenous malformation (AVM) of the upper lip is reported. A 47-year-old man complained of swelling and spontaneous bleeding of the right upper lip, without pain. Physical examination showed a 10×20mm palpable soft mass in the lesion. Angiograms of the external carotid arteries revealed a diffuse AVM involving the right upper lip. The AVM was supplied by both facial arteries. Superselective embolization of the feeding arteries was performed preoperatively. Surgical excision of the AVM was done simultaneously. Blood loss was 65ml. The patient remained asymptomatic 34months after the procedures.