A 26-year-old male presented with gradually worsening quadriparesis over 3 weeks and decreased respiratory drive over 2 days. MRI showed cord swelling and central hyperintensity on T2 images, resembling central grey matter. Contrast MRI showed dilated enhanced perimedullary veins on the spinal cord surface. The final diagnosis was an intracranial DAVF fed by the meningohypophyseal trunk and middle meningeal artery with spinal perimedullary venous drainage. Treatment with occlusion of the fistula using Onyx liquid embolic agent was planned.