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The authors present a case of optic nerve glioblastoma that was treated with subtotal resection followed by adjuvant radiation therapy and temozolomide. One year following the initial diagnosis, the patient developed a right cerebellar lesion, which was histopathologically consistent with glioblastoma. This case represents the first report of transtentorial dissemination of an optic nerve glioblastoma. In addition, the authors reviewed the literature regarding optic nerve glioblastomas. Of the 73 previously reported cases of malignant optic nerve gliomas, 32 were histologically confirmed glioblastomas. The mean age at diagnosis was 62 years, and 56% were male; the median survival was 7 months. A malignant glioma of the optic nerve should be considered in the differential diagnosis of a patient with rapidly progressive visual loss. 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