Perimedullary arteriovenous fistula (AVF) has shunt on the spinal cord surface and it can be treated with surgery, or endovascular embolization. Intramedullary arteriovenous malformation (AVM) has its nidus in the cord and is difficult to treat either by surgery or endovascular technique. We report our experience with endovascular embolization in the treatment of perimedullary AVF and intramedullary AVM. Four consecutive cases of spinal perimedullary AVF and six intramedullary AVM were retrospectively reviewed. These cases were evaluated and treated at Beijing Tiantan Hospital, China, over a 2-year period. A review of previously reported cases was also performed. There was no significant difference in the presentations (P = 0.348), level of lesions (P = 0.350), arterial suppliers (P = 0.801), and associated vascular anomalies between perimedullary AVF and intramedullary AVM, except for age (P = 0.014) and treatment modalities (P = 0.003). The patients with perimedullary AVFs were younger in our cases; the age at onset of symptoms averaged 25.8 years compared to 31.7 years for intramedullary AVMs. There was a significant male predominance for both lesions, and a significantly higher incidence of subarachnoid hemorrhage than in spinal dural AVFs. Regarding treatment, endovascular coil embolization is frequently used in perimedullary AVF and liquid embolic agent is an effective therapeutic choice in intramedullary AVM. Perimedullary AVF and intramedullary AVM are dissimilar with dural AVF in clinical characteristics. Our experience suggests that the endovascular treatment of spine perimedullary AVFs and intramedullary AVMs is feasible and effective. Endovascular treatment for intramedullary AVMs is still challenging, the main problem is acute ischemia injury of the spinal cord.