Recent advances in neuro-ophthalmology have been made in both diagnosis and therapy. New discoveries in molecular genetics have resulted in the identification of abnormalities in the nuclear and mitochondrial genomes likely responsible for retinitis pigmentosa and Leber's hereditary optic neuropathy, respectively. Neuroimaging continues to broaden our diagnostic abilities in both optic nerve disease and lesions of the retrochiasmal visual pathways. The role of steroids in idiopathic optic neuritis has been clarified by the Optic Neuritis Treatment Trial. The optic nerve sheath decompression procedure has become a useful and effective adjunct in the management of visual loss secondary to pseudotumor cerebri, and is currently being evaluated as a potential treatment for anterior ischemic optic neuropathy.