Abstract. Spinal cord perimedullary arteriovenous fistulas (PMAVFs) are rare and belong to type IV spinal cord arteriovenous malformations (AVMs). Little is known regarding the treatment and prognosis of spinal cord PMAVFs. In the present study the relevant literature from PubMed was reviewed, and it was found that these fistulas can occur at all ages but are more common in children. In children, most spinal cord PMAVFs are large and with high flow, begin with bleeding and are frequently associated with hereditary hemorrhagic telangiectasia. However, in adults, most spinal cord PMAVFs are small and with low flow and begin with progressive spinal cord dysfunction. The early diagnosis of spinal cord PMAVFs is generally difficult, and symptoms can be very severe at the time of diagnosis. Digital subtraction angiography remains the gold standard; however, computed tomography angiography and magnetic resonance angiography are also promising. Spinal cord PMAVFs can be treated by endovascular embolization, surgical removal or a combination of the two methods. Most spinal cord PMAVFs show good outcomes after the appropriate treatment, and the prognosis is primarily associated with the blood flow of the PMAVF. For high-flow spinal cord PMAVFs, endovascular embolization is more effective and can lead to a good outcome; however, for low-flow spinal cord PMAVFs, surgical removal or the combination with endovascular embolization is the optimal choice. The prognosis for low-flow types is slightly worse than for high-flow spinal cord PMAVFs in children, but the outcome is acceptable.