Teratomas account for 3% of all childhood tumors, with the majority occurring in the sacrococcygeal region and in the ovary. Intradural spinal teratomas are extremely rare dysembryogenetic tumors. Spinal cord teratomas may be extradural, intradural or intramedullary. Intramedullary ones are the least frequently seen. We have extensively reviewed the literature for intramedullary spinal cord teratomas in children. Although an intramedullary teratoma of the conus medullaris in children is a rare entity, it should be considered in the differential diagnosis of masses involving the conus medullaris.