Spinal atypical teratoid/rhabdoid tumor (AT/RT) is extremely rare. We report this rare case and review the literature of spinal AT/RT. A 10-month-old girl presented with rapidly progressive paraplegia. Magnetic resonance images revealed an intramedullary tumor occupying the entire spinal canal below Th10. An urgent operation, consisting of decompression by laminectomy and biopsy, was performed. Histologically, the tumor specimen had many rhabdoid cells with prominent nuclei and eosinophilic cytoplasmic inclusion. It showed mitosis and necrosis. The tumor cells were immunoreactive for vimentin, desmin, smooth muscle actin, neuron-specific enolase, neurofilament, epithelial membrane antigen, and CAM5.2. Despite chemotherapy and radiotherapy, she died 3 months after admission. The present case is only the third detailed report of spinal AT/RT. Spinal AT/RT carries a poor prognosis, and therefore should be distinguished from other embryonal tumor.