Primary spinal atypical teratoid/rhabdoid tumor (AT/RT) is extremely rare. A 9-month-old boy presented with tetraparesis. Subsequent MRI showed an intradural extramedullary bulky mass extending from C4 to T6. Subtotal removal of the tumor was performed and spinal AT/RT was proven histologically. Despite chemotherapy and radiation therapy, leptomeningeal dissemination was found on follow-up MRI. The patient died 20 months after his initial symptoms. We describe the MRI findings of this case and review the literature describing this rare disease.