Pleomorphic xanthoastrocytoma (PXA), an uncommon glial neoplasm, typically affects adolescents and young adults and accounts for less than 1% of all astrocytic neoplasms. The authors present a case of PXA located entirely in the third ventricle in a 24-year-old male patient. The patient presented with a 6-month history of headaches, progressive decline in cognitive function and profound behavioral disturbances. He was admitted to the hospital with signs of increased intracranial pressure. Magnetic resonance imaging showed a well-enhanced solid tumor, located entirely inside the third ventricle. The tumor was totally removed via a right fronto-pterional trans-lamina terminalis approach and neuropathology report confirmed PXA diagnosis. No further treatment was indicated. To the best of our knowledge, this is the first case report of a solid PXA confined to the third ventricle.