Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant central nervous system neoplasm usually seen in young children and infants. Prognosis for AT/RT is poor, with most patients dying within 1 year of presentation. AT/RT most commonly occurs intracranially. Location in the spine, though previously reported, is rare, and imaging findings have not been emphasized in the past. We present a case of AT/RT occurring in the thoracolumbar spine of a child and review available clinical and imaging findings in previously reported cases of spinal AT/RT.