Central nervous system atypical teratoid/rhabdoid tumor(CNS rhabdoid tumor) is a rare malignancy of uncertain origin. It typically occurs in infants and young children and comprises only a small fraction of pediatrics CNS malignancies. The tumor contains a large spindled cell component as classical rhabdoid morphology and focal areas resembling primitive neuroectodermal tumor. The tumor is defined histopathologically by the presence of rhabdoid cells, but contains considerable heterogeneity of the cell type, including the frequent presence of primitive neuroectodermal tumor. The prognosis for children with CNS rhabdoid tumor is dismal. We experienced a case of a three-year-old female who had been transferred to our hospital for seizure and vomiting. She was diagnosed as CNS atypical teratoid/rhabdoid tumor by biopsy. In spite of surgery and intensive postoperative multi-agents chemotherapy, she developed a local recurrence around the operation site at six months after surgery. We present this case with a brief review of related literatures.