Pediatric spinal perimedullary arteriovenous fistula (PMAVF) is rare but may cause permanent disability. We aim to summarize the clinical features of pediatric PMAVFs and our clinical experience in their treatment and to evaluate the effect of endovascular treatment in a large cohort. From 2008 to 2017, 51 PMAVFs in pediatric patients (<14 years' old) treated with endovascular techniques were retrospectively reviewed, including 24 type IVb (47.1%) and 27 type IVc (52.9%) lesions. Clinical features, radiological findings, treatment, and outcomes were evaluated. Thirty-eight boys and thirteen girls were included, and the mean age at presentation was 5.6±4.1 years. Acute neurological deterioration was identified in 33 patients, and 21 of those patients (63.6%) suffered from bleeding. The annual bleeding rate before treatment was 2.55%. After transarterial embolization with coils and glue, 46 PMAVFs (90.2%) were completely occluded, and five (9.8%) were obliterated by supplementary microsurgery. During a follow-up period of 6 to 119 months (mean 58.4±16.7 months), the patients' clinical states were improved in 42 cases (82.4%), stationary in nine cases (17.6%), and aggravated in none. Type IVc patients had a longer preoperative period, more chronic symptoms, a lower cure rate by embolization, and less improvement of symptoms than type IVb patients had (P<0.05). Pediatric PMAVF is a special subgroup of intradural arteriovenous shunt that should be treated early. Endovascular embolization is safe and effective in the treatment of pediatric PMAVFs. © Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.