Many neuro-ophthalmic diseases have a clear sex predilection, which is important to recognize in making the diagnosis based on risk stratification and understanding the pathogenesis of the disease. This review discusses the more common neuro-ophthalmic diseases with a female predilection, including idiopathic intracranial hypertension, cerebral venous sinus thrombosis, meningioma, multiple sclerosis, migraine, breast-cancer associated neuro-ophthalmic manifestations, sarcoidosis, bisphosphonate-associated orbital inflammation, and pregnancy-related neuro-ophthalmic disorders. In addition, the male predominance in the clinical manifestation of Leber's Hereditary Optic Neuropathy is discussed. Lastly, the etiology of the sex discrepancies for each disease is explored.