Atypical teratoid/rhabdoid tumor (AT/RT) of spine usually reported to develop in the brain, while it rarely manifest in the spine. It consists of rhabdoid cells and is highly malignant. AT/RT appears at various sites throughout the body, such as in the central nervous system, liver, kidneys, abdomen, and soft tissues. Among them, spinal AT/RT is rare, and AT/RT of lumbar spine is extremely rare; only a few cases have been reported. We present the case of an AT/RT of lumbar spine in a 16-month-old boy.
