Atypical teratoid/rhabdoid tumor is a highly malignant tumor of the central nervous system, usually occurring in the posterior fossa in infants and young children. Supratentorial example is relatively rare, especially with involvement of the cerebral ventricle system. Herein, we reported 2 cases of atypical teratoid/rhabdoid tumor located in the septum pellucidum within the lateral and third ventricles and right parietooccipital region, respectively. Histopathologically, both of the tumors were composed of rhabdoid tumor cells and mesenchymal components, without primitive neuroectodermal tumor or epithelial differentiation. Immunohistochemical staining showed that these tumor cells reacted positively for vimentin, S-100 protein, synaptophysin, and neuron-specific enolase. Only 1 case was found to be epithelial membrane antigen reactive. The tumor cells lacked nuclear expression of INI1. These cases emphasize that atypical teratoid/rhabdoid tumor should be also considered in the list of differential diagnosis, even when these rhabdoid tumor cells do not arise in the predilection sites.