A 45-year-old female patient presented with symptoms of polydipsia and polyuria, menopause, headache, gait disturbance and deteriorated mental state. Brain magnetic resonance imaging (MRI) showed an irregular mass in the anterior third ventricle. The tumor was excised using a transfrontal approach from the anterior section of the third ventricle. The histological diagnosis was of an intracranial pure yolk sac tumor. The patient underwent radiotherapy and suffered no tumor recurrence one year after the surgery. Overall, when heterogeneous enhancement and an irregular mass with surrounding invasion and ventricular dilation are observed in the anterior third ventricle of an adult, a yolk sac tumor should be considered, and MRI may aid the differential diagnosis. A combination of surgical resection and radiotherapy is recommended for the yolk sac tumor.