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Neuro-ophthalmological emergencies are some ocular manifestations, which are associated with the sight or life-threatening diseases if not promptly treated, including acute visual loss, sudden diplopia or anisocoria.Some severe nerve system or orbitocranial inflammatory diseases frequently present neuro-ophthalmological symptoms at the early stage.Acute visual loss is the common complaint of optic neuritis, arteritic anterior ischemic optic neuropathy, rhino-orbital-cerebral mucormycosis, and pituitary apoplexy.Diplopia usually occurs in cavernous sinus thrombosis or intracranial aneurysm because of cranial nerve palsies.Additionally, intracranial aneurysms and carotid artery dissecting aneurysm often present anisocoria initially.The clinical symptoms, signs, diagnosis and management of these disorders were reviewed from the point of emergency medicine, and the role of neuro-ophthalmological signs in the diagnosis was highlighted in this review, aiming to help ophthalmologists improve the awareness of these conditions, and emphasize the urgency of these signs.