We report a case of anaplastic ganglioglioma. A 45-yr-old woman was admitted with a 5-month history of headache and dizziness, both of which progressed slowly. Preoperative magnetic resonance imaging revealed a strong enhancing mass in the left frontal lobe extending to the cingulate gyrus. Adjuvant radiation therapy and chemotherapy were given after gross total resection of the tumor. Histological and immunohistochemical studies showed an anaplastic ganglioglioma. Gangliogliomas of the central nervous system are rather uncommon tumors, and anaplastic ones are extremely rare. The pertinent literature regarding gangliogliomas is reviewed.