Atypical teratoid/rhabdoid tumor (ATRT) is usually seen in children and is usually located intracranially; it generally has a poor prognosis. Due to this tumor's rarity and the lack of randomized controlled trials, it has been challenging to define optimal therapy and to make treatment advances. Treatment options are surgery, chemotherapy, and radiotherapy. This is a case report of a man with spinal ATRT, aged 44 years, who was treated with radiotherapy.