We report 2 cases of primary central nervous system (CNS) lymphoma arising in the region of the optic nerve. For both patients, diagnosis of lymphoma was impossible without histological examination because of the rarity of the lymphoma location. The first case involved an 84-year-old woman who developed loss of vision and hypopituitarism. Intraoperative finding was optic glioma; histological diagnosis was diffuse large B cell lymphoma, however. The second case involved a 67-year-old man who developed loss of vision. The pre-surgical diagnosis was optic nerve neuritis; this was then revised to granuloma. The tumor arose in the optic nerve. Methotrexate and rituximab were administered and the patient remained in complete remission for 3 years. However, a sudden intratumoral hemorrhage occurred. Although most of the lymphoma cells obtained from the initial surgery were negative for vascular endothelial growth factor (VEGF) immunoreactivity, high levels of VEGF immunoreactivity in lymphoma cells was detected in the specimen obtained after intratumoral bleeding at recurrence, and correlation between VEGF reactivity and tumor recurrence was suggested. To date, primary CNS lymphomas with intracerebral hemorrhage have been reported in 3 cases only, and a correlation between intratumoral hemorrhage and the degree of VEGF expression has been suggested. VEGF also might have predictive significance for recurrence.