This study reports the case of a 75-year-old woman with intermittent speech disturbance, headache, and general malaise. Brain magnetic resonance imaging showed a double lesion located in the third ventricle and temporal horn of the lateral ventricle. We completely removed the lesion in the third ventricle, adhering to the septum pellucidum. The histopathological diagnosis was Burkitt lymphoma, which was confirmed to be primary in postoperative investigation. To our knowledge, this is the first case of primary Burkitt lymphoma involving cerebral ventricles. Finally, we reviewed the characteristics of primary central nervous system Burkitt lymphoma.