A Malignant rhabdoid tumor (MRT) arising in the right temporoparietal lobe of a 9-year-old boy is described along with the results of an immunohistochemical study. The patient initially sought medical attention for a ptosis and right sided headache. The child underwent a subtotal resection of the tumor, followed by radiotherapy and systemic chemotherapy, but died three years after surgery. A MRT, a primary neoplasm of the central nervous system (CNS), is an entity of unknown histogenesis with a dismal prognosis, which only occurs in early childhood. Histologically similar tumors with more varied morphological features have been designated as an atypical teratoid/rhabdoid tumor. However, a classical MRT is extremely rare in the CNS and our case represents a classical CNS MRT.