Congenital arteriovenous malformations (AVMs) involving the pelvis or an extremity were occluded in 16 symptomatic patients, who subsequently underwent tailored embolotherapy. An additional 11 patients did not undergo embolization due to unfavorable vascular anatomy or lack of significant symptoms. Permanent occlusive agents including isobutyl cyanoacrylate, particles of polyvinyl alcohol foam, and coils were used to embolize the multiple feeding vessels and, when possible, the nidus of the AVM. All patients experienced dramatic reduction in pain and resolution of ulceration and bleeding, with a mean follow-up period of 41 months. Symptoms recurred in four patients but again resolved with repeat embolization. The authors conclude that selective and repetitive embolization is highly effective in palliation of symptomatic congenital AVMs.