The authors describe 3 cases of cerebral pleomorphic xanthoastrocytoma, one of which has an unusual neuroradiological appearance, and review the clinical and prognostic features of the 96 cases reported in the literature. Pleomorphic xanthoastrocytoma is a neuroectodermal tumor that affects young patients, in a superficial or supratentorial site, most frequently at the temporal level. It is difficult to formulate a differential diagnosis with other neuroectodermal tumors or with meningioma on the basis et neuroradiological appearance, as demonstrated by one of the cases reported here. The prognosis of this tumor is very good, regardless of the type of treatment performed, although both relapse and, more rarely, malignant tumor evolution are possible.