Cerebellar pleomorphic xanthoastrocytoma (PXA) is a rare tumor. The most common manifestation of PXA is a seizure, as opposed to the headache and dizziness that were present in our reported case; these atypical symptoms were related to the development of tumor in cerebellum. We describe here a case of PXA in the cerebellum of a 49-year-old female and we discuss the radiological, histological and immunohistochemical findings of PXA that occurred in the cerebellum.