INTRODUCTION: The aim of this study was to report a rare pediatric case of spontaneous spinal epidural hematoma (SSEH) mimicking Guillain-Barré syndrome (GBS), secondary to an epidural arteriovenous malformation (AVM). Furthermore, a case-based update and insight into the entity is attempted.METHODS: An 8-year-old male presented with progressing severe lower limb weakness and no traumatic history. Presentation was mimicking GBS with ascending symptoms. Magnetic resonance (MR) scan revealed a dorsal epidural mass, extending from C6-C7 to T2, compressing the spinal cord. Emergency laminoplasties and surgical evacuation of the hematoma were performed. An up-to-date review of reported SSEH cases in children was conducted, with emphasis on underlying vascular malformations (epidural AVMs in particular). Pathogenesis, predisposing factors, imaging, diagnosis, treatment and outcome are discussed.RESULTS: The hematoma was successfully evacuated. A vascular membrane on the dura was peeled off and sent for histopathology. There was no evidence of intradural vascular penetration. The patient improved postoperatively and was able to walk with support 7 months later. Histology revealed closely packed thin-walled angiomatous structures with wide lumens (filled with red blood cells) with walls composed of collagen and smooth muscle fibers, findings consistent with AVM.CONCLUSIONS: Non-traumatic SSEH is rare in the pediatric population. Although vascular malformations are suspected, they are extremely rarely identified histopathologically. This case represents one of the very few reports of pediatric SSEH caused by a histologically proven, purely epidural AVM. High index of clinical suspicion and low threshold for MR can lead to timely diagnosis and prompt treatment with good functional outcome.