Pleomorphic xanthoastrocytoma (PXA) has been considered as a low grade tumor of adolescents and young adults. Although this tumor often shows cystic component, the hemorrhage within the cyst is extremely rare. The authors report a rare case of cystic PXA with a hemorrhage within the cyst and the mural nodule in the left frontal lobe. A 64-year-old male presented with a week history of the right side hemiparesis. After gross total resection of the tumor, the patient was fully recovered from neurological deficit. It is suggested that this typically benign tumor could be presented with hemorrhage, causing a rapid neurological deterioration. Prompt surgical intervention, especially total removal of the tumor can provide an excellent functional recovery.