A 28-year-old woman with a complex peripheral congenital arteriovenous malformation in the left shoulder and left upper arm was treated by a combination of surgery and embolization. In the consecutive four operations, the major feeding arteries of the arteriovenous malformation were ligated, but as her complaints increased, transcatheter embolization was planned for releaving the symptoms in the first aspect. A total of eight embolization sessions were performed with Onyx during the 3-year period. The arteriovenous communications were occluded by embolization, with approximately more than an 80% decrease in the lesion size angiographically. After these interventions, the patient's symptoms improved clinically and dyspnea was lessened. Peripheral congenital arteriovenous malformation is a rare and therapeutically challenging condition in which a combination of surgery and embolization would be necessary to obtain a good result.