The authors report a case of pleomorphic xanthoastrocytoma (PXA) with neurofibromatosis type 1 (NF-1). A 21-year-old woman, noted to have had a number of café au lait spots since birth, presented with the chief complaint of headache. Neuroradiological studies revealed a cystic tumor of the left parietal lobe. Surgical treatment was performed. The tumor was located on the surface of the parietal lobe with the formation of cysts. Light, electron microscopic, and immunohistochemical examination showed the characteristics of typical PXA. This is the second reported case of NF-1 associated with PXA.