A 3-year-old boy presented to our hospital with progressive neurological deficits. Spinal magnetic resonance imaging (MRI) revealed a perimedullary macro-arteriovenous fistula (PMAVF) resulting in a large venous pouch within the parenchyma of the lower cervical spinal cord. Transarterial varix embolization of the fistula from the venous side was performed using N-butyl cyanoacrylate with tantalum powder. Postembolization angiography confirmed obliteration of the fistula, and MRI revealed thrombosis and reduction in size of the venous component. The patient's clinical symptoms were reduced dramatically following the intervention, and no neurological complications occurred due to the treatment. The prognosis of spinal PMAVF depends primarily on the presence of medullar signs and symptoms and on time to treatment. The volume of the venous pouch after the intervention was markedly reduced, and complete clinical recovery was obtained. Urgent endovascular intervention, as seen in this case, is considered first-line therapy and aims to decrease the risk of neurological sequela.