The objective of this study was to review our pediatric series of perimedullary arteriovenous fistula (PMAVF), investigating the clinical characteristics, angioarchitecture, treatment strategy, and outcome of PMAVF in pediatric patients. Nineteen children with PMAVF treated in Xuanwu Hospital between 1986 and 2007 were reviewed. The demographic data, clinical symptoms, angiographical characteristics, classification, treatment modality, and outcome were compiled and analyzed. Clinical status was evaluated according to the modified Aminoff and Logue scale. In our pediatric series of PMAVF, the mean age of diagnosis was 8.1 years (range, 0.8-13.7 years). Male predominance was found in our series (M/F ratio, 3.75:1). Eleven children (57.9%) presented with acute onset of symptoms, and eight cases (42.1%) had progressive evolution of the diseases. In 13 cases (68.4%), the fistulas were classified as type III, with type II in six cases. Four lesions (21.1%) were located in the cervical region, with 15 lesions (78.9%) in thoracolumbar region. Ten cases (II = 2, III = 8) were treated with embolization, and nine (II = 4, III = 5) with combined embolization and complementary surgery. According to the criteria of therapeutic effectiveness, 31.6% of cases (6/19) were cured, and nine patients (47.4%) had improved. PMAVFs in pediatric population exhibit many characteristics in clinical and angiographical aspects, need for special considerations. Most of PMAVFs in pediatric patients are high-flow fistulas, and endovascular embolization should be the first choice of treatment. After appropriate therapy, most of children can get satisfactory outcomes.