To characterize the clinical presentation, imaging features and endovascular treatment of paraspinal non-vertebral arteriovenous fistulas along the segmental nerve. Retrospective review was performed on the five patients identified in our database covering 1985 to 2003. All patients presented with an incidentally found continuous murmur over the upper paraspinal or parasternal regions before three years old. In four patients, the AV fistula was in the mid-thoracic level and at L3 in one. All AV fistulas were a high-flow single-hole fistula at the neural foramen with venous drainage into paraspinal and epidural veins without intradural reflux. All fistulas were endovascularly occluded in the same session as the diagnostic angiography. The fistula was occluded with detachable coils in one case and with N-butylcyanoacrylate (NBCA) with flow control in four cases. Complete occlusion of the fistula was obtained in all cases and all patients remained neurologically intact at the last follow up (average six years). Non-vertebral paraspinal arteriovenous fistula along the segmental nerve is a specific disease entity seen in children. Embolization is the first choice of treatment for this disease.