A young patient with pleomorphic xanthoastrocytoma (PXA), a usually benign cerebral tumor, had two recurrences in a short time period. The clinical, pathological and neuroradiological features, including PET with [18F]-fluorodeoxyglucose (FDG), are presented. The PET-FDG study revealed the recurrent tumor to be hypermetabolic. The diagnosis was confirmed histopathologically. As the clinical outcome of patients harboring PXA is not easy to predict because of possible recurrence and/or transformation into more aggressive gliomas, we discuss the predictive indicators of more aggressive clinical behavior.