Purpose: We report the clinical features, diagnoses, and treatments of patients with neuro-ophthalmological diseases presenting to a tertiary referral center emergency room (ER) of South Korea. Methods: We retrospectively analyzed the medical records of 205 patients who visited the ER of the Asan Medical Center and then were referred to our neuro-ophthalmology department from May 2020 to April 2022. The initial diagnoses of ophthalmology residents were compared to the final diagnoses of neuro-ophthalmologists. We describe the symptoms, diagnoses, and treatments. Results: The median time from the onset of symptoms to the ER visit was 3 days; diplopia (49.1%) and vision loss/a visual field defect (38.9%) were the chief complaints. The most common causes of diplopia were isolated cranial nerve 4 (37.5%), 6 (22.9%), and 3 palsy (18.4%) and supranuclear palsy (9.2%). In patients with vision loss/visual field defect, the most common causes were optic (20.5%), ischemic (17.1%), and compressive (8.0%) optic neuropathies. For 121 cases (59.0%), multidisciplinary consultations with the departments of neurology, neurosurgery, and internal medicine were scheduled. After initial management in the ER, 38 diagnoses (18.5%) changed after evaluation by neuro-ophthalmologists. Conclusions Over half of all neuro-ophthalmology patients presenting to an ER required comprehensive interdisciplinary evaluation and over a third required inpatient treatment.