A report of successful combined endovascular and surgical management of an unusual case of metameric (juvenile) spinal arteriovenous malformation (AVM) is presented. The malformation had extradural and paraspinal components, but no intradural elements. It had caused rapid neurological deterioration to near-complete paraplegia prior to treatment (American Spinal Injury Association [ASIA] Grade C). A combination of endovascular occlusion of major feeding vessels and excision of the malformation resulted in a complete neurological recovery (ASIA Grade E). The authors conclude that selected metameric AVMs can be successfully treated with multimodal therapy. This case further illustrates the fact that not all spinal vascular malformations are easily categorized.