Two out of three patients with pleomorphic xanthoastrocytoma were initially misdiagnosed and correctly interpreted only at tumour recurrence, with progression to malignancy in one. The third patient presented with a remarkably long history of epilepsy. Pleomorphic xanthoastrocytoma is a low grade astrocytoma that is still confused with other tumours. Because pleomorphic xanthoastrocytoma can become malignant even after many years of benign behaviour, a long term follow up is necessary.