Four cases of pleomorphic xanthoastrocytoma (PXA) were collected from among 688 glioma patients who underwent operation at the Institute of Neurosurgery, University of Naples "Federico II" between January 1973 and December 1994. Three were females and one male, ranging in age from 10 months to 65 years. Three tumors were superficial in location, appearing as a meningo-cerebral mass in the temporo-parietal region. In one case, the tumor was situated deep within the brain (capsulo-thalamic region), without contact with leptomeninges. Three patients had experienced epileptic seizures, whereas one patient presented with an ictal episode. Tumor excision was grossly total in two cases, and subtotal in the remaining two. In three cases, histological examination demonstrated a "typical" PXA; conversely one tumor (subtotal excised) was an "atypical" PXA. The two patients with incomplete surgical resection were postoperatively treated with fractionated brain radiation therapy. Of the two patients who had grossly total removals, one showed tumor recurrence 6 years after surgery, and underwent operation (the recurrent neoplasm did not exhibit malignant transformation); the second patient was free of tumor at 14 months following craniotomy. Of the two patients who had undergone subtotal removals, one died because of massive regrowth of the lesion 22 months after surgery, whereas the second patient was asymptomatic at 1 year follow up.