Purpose: We aim to evaluate the clinical characteristics, etiologies, and their associated variables of the common neuro-ophthalmologic conditions affecting the pediatric patient who presents to the emergency department (ED) with eye complaints. Methods: We identified the 45 children who visited an ED for ophthalmologic problems and performed the neurologic evaluation between 2009 and 2012. The patients with traumatic injury or topical ocular disease were excluded. The medical records of patients were retrospectively reviewed. Results: The mean age of the 45 children (boy: 25, girl: 20) was 8.0±4.7 (range, 0.04–17) years. The main ophthalmologic problems included decreased visual acuity in 17 children (37.8%), diplopia in 13 (28.9%), ptosis in 13 (28.9%), ocular pain in 6 (13.3%), and nystagmus in 4 (8.9%). Other conmitant symptoms consisted of headache in 16 (35.6%), dizziness in 4 (8.9%), nausea/vomiting in 3 (6.7%), ataxia in 3 (6.7%), and fever in 3 (6.7%). Underlying etiologies were revealed as followings; myasthenia gravis (8/45, 17.8%), migraine (8/45, 17.8%), optic neuritis (7/45, 15.6%), CNS infections or inflammation (6/45, 13.3%), Miller-fisher syndrome (MFS) (3/45, 6.7%), isolated nerve palsy (3/45, 6.7%), tumor (2/45, 4.4%), congenital malformation (2/45, 4.4%), and tic disorder (1/45, 2.2%). Biocular diplopia was common initial symptom of the children with MFS or isolated nerve palsy. Diplopia or ptosis was noted in the children with a tumor. Conclusion: It is not easy to provide a correlation between the neuro-ophthalmologic symptoms and many etiologies. Careful neurologic examination and neuroimaging are essential in the evaluation of pediatric neuro-ophthalmologic diseases.