Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) of infancy and childhood is a unique histological entity with an extremely aggressive natural history. In spite of multiple treatment regimens consisting of maximal surgical resection, radiation therapy, and multiagent aggressive chemotherapy, the prognosis is very poor. Here in this study we analyze the charts, presenting symptoms, imaging studies, surgical procedures and adjuvant therapy modalities of 10 AT/RT patients between 1995 and 2004.