A case of a histologically proven rhabdoid tumor of the cervical spine in a 19-year-old Caucasian male is presented. Primary extrarenal rhabdoid tumors are very rare. When the central nervous system is involved, the tumor usually is located in the brain. Only three cases of primary spinal rhabdoid tumor have been reported. This case is the first reported extradural rhabdoid tumor of the spinal canal and the first case of a rhabdoid tumor located in the spinal canal with bony involvement.