An atypical teratoid rhabdoid tumor (AT/RT) is a rare tumor usually diagnosed in childhood. Although usually a brain tumor, AT/RT can occur anywhere in the central nervous system (CNS), including the spinal cord. About 60% will be in the posterior cranial fossa (particularly the cerebellum). One review estimated 52% in the posterior fossa, 39% are supratentorial primitive neuroectodermal tumors (sPNET), 5% are in the pineal, 2% are spinal, and 2% are multifocal. In the United States, three children per 1,000,000 or around 30 new AT/RT cases are diagnosed each year. AT/RT represents around 3% of pediatric cancers of the CNS. Around 17% of all pediatric cancers involve the CNS, making these cancers the most common childhood solid tumor. The survival rate for CNS tumors is around 60%. Pediatric brain cancer is the second-leading cause of childhood cancer death, just after leukemia. Recent trends suggest that the rate of overall CNS tumor diagnosis is increasing by about 2.7% per year. As diagnostic techniques using genetic markers improve and are used more often, the proportion of AT/RT diagnoses is expected to increase. AT/RT was only recognized as an entity in 1996 and added to the World Health Organization Brain Tumor Classification in 2000 (Grade IV). The relatively recent classification and rarity has contributed to initial misdiagnosis and nonoptimal therapy. This has led to a historically poor prognosis. Current research is focusing on using chemotherapy protocols that are effective against rhabdomyosarcoma in combination with surgery and radiation therapy. Recent studies using multimodal therapy have shown significantly improved survival data. In 2008, the Dana-Farber Cancer Institute in Boston reported two-year overall survival of 53% and event-free survival of 70% (median age at diagnosis of 26 months). In 2013, the Medical University of Vienna reported five-year overall survival of 100%, and event-free survival of 89% (median age at diagnosis of 24 months). Survival rates can be significantly improved when the correct genetic diagnosis is made at the outset, followed with specific multimodal treatment. Signs and symptoms Clinical signs and symptoms depend on the location of the tumor. Since many of the tumors occur in the posterior fossa, they present like other posterior fossa tumors, often with headache, vomiting, lethargy, and ataxia (unsteady gait). A case of a seven-month-old child with a primarily spinal tumor that presented with progressive paraplegia and abnormal feeling in the legs was reported. Genetics Genetic similarities have been found within rhabdoid