The authors report the case of a 36-year-old woman who underwent gross total resection of a right cerebellar pleomorphic xanthoastrocytoma with atypical features. She had undergone surgery 16 years previously for what was thought to be a right frontal glioblastoma multiforme. In retrospect, based on the histopathology and the clinical course, both lesions were considered to represent atypical variants of pleomorphic xanthoastrocytoma. This report examines the histological and clinical characteristics of this posterior fossa lesion, which exhibited histologically malignant features but has run a relatively indolent course.