Spinal epidural arteriovenous malformations (AVMs) are rare diseases. In a pediatric emergency setting, spinal epidural AVMs should be considered as part of the differential diagnosis for acute extremity weakness and numbness. The authors report herein a case of spinal cord ischemia and spontaneous spinal epidural hematoma (SSEH) arising from an epidural AVM. A 13-year- old adolescent boy presented with recurrent severe interscapular pain followed by complete motor and sensory paralysis. The magnetic resonance imagingT2 sequences demonstrated increased signal within the bilateral anterior horns involving the anterior spinal artery territory. The patient was misdiagnosed as having transverse myelitis until epidural hematoma occurred and postoperative pathological studies confirmed an AVM. AVMs are recognized as 1 reason for spinal cord ischemia and have been identified as underlying lesion for SSEH Howell et al. (1987), Aminoff et al. (1974) [ 1,2]. Although suspected, there were no vascular malformations identified as the underlying lesion for spinal cord ischemia and hematoma in the same patient. To our knowledge, this represents the first case of spinal cord ischemia and SSEH caused by a histologically proven epidural AVM.