Neuro-ophthalmological emergencies constitute vision or life-threatening conditions if diagnosis and treatment are not promptly undertaken. Even with immediate therapy, these clinical entities carry a high rate of morbidity. They may present with diplopia, visual loss, and/or anisocoria. Arteritic anterior ischemic optic neuropathy is an ominous condition, which can cause permanent and severe vision loss, stroke, or aortic dissection, requiring immediate steroid therapy. Pituitary apoplexy may go unnoticed if only computed axial tomography is performed. Diseases affecting the cavernous sinus and orbital apex region, such as cavernous sinus thrombosis or mucormycosis, can give rise to simultaneous vision loss and diplopia and, if not treated, may extend to the brain parenchyma causing permanent neurological sequela. An isolated third nerve palsy may be the harbinger of a cerebral aneurysm, carrying a significant risk of mortality. Horner syndrome can be the initial presentation of a carotid dissection, an important cause of stroke in the young adult. The neurohospitalist should be familiar with the workup and management of neuroophthalmological emergencies.