Identifying a source of spontaneous subarachnoid hemorrhage (SAH) or intraventricular hemorrhage (IVH) in patients with negative results on cranial angiographic imaging can be a diagnostic challenge. The authors present the case of a 14-month-old girl who presented with lethargy and spontaneous SAH and IVH, and later became acutely paraplegic. Except for the SAH and IVH, findings on neuroimages of the brain were normal. Magnetic resonance imaging revealed an intramedullary thoracolumbar spinal cord hemorrhage that was found to be associated with arterialized veins intraoperatively. Catheter-based diagnostic angiography identified a spinal perimedullary macroarteriovenous fistula (macro-AVF) that was completely embolized with Onyx, negating the need for further surgical intervention. The authors believe this to be the first reported case of a thoracolumbar perimedullary macro-AVF presenting with SAH and IVH. In addition, descriptions of Onyx embolization of a spinal AVF in the literature are rare, especially in pediatric patients.