Pleomorphic xanthoastrocytoma (PXA) is a rare benign tumor that is usually located in the superficial cerebral hemisphere.Most reports of PXAs have included only a single case or small series.Therefore,the data with respect to the natural history of this tumor are fragmentary.We report a case of a PXA in the unusual location of the right lateral ventricle with extensive subarachnoid dissemination.To our knowledge,this is a rare case of PXA in the lateral ventricle.In addition,extensive subarachnoid space dissemination of this distinctly benign type of glioma is exceedingly rare.In our case,there was meningeal dissemination and metastases to the bilateral trigeminal nerves and oculomotor nerves.The neuroradiographic features,tumor location,and dissemination were reviewed.