PURPOSE: To evaluate the role of the ophthalmologist at presentation and during follow-up of children with arterial stroke. METHODS: This retrospective case series comprised 26 children with arterial stroke who were followed for at least 12 months in our tertiary pediatric medical center in 2005-2015. Demographic data and findings on radiological neuroimaging, ophthalmological and neurological examination were retrieved from the patient medical files. RESULTS: Of the 26 children identified with stroke, 5 (20%) had a metabolic syndrome, 3 (11%) cardiac anomaly, 3 (11%) recent major surgery for repair of cardiac anomaly, 3 (11%) vascular anomaly, 3 (11%), head trauma with traumatic dissection, 1 (4%) had hypercoagulability and in 8 patients (31%), no apparent cause was found (idiopathic). Only 9 (35%) had an eye exam during the follow-up. Eleven patients had a nonophthalmological neurological deficit as a result of the stroke. Positive ophthalmological manifestations included hemianopic visual field defect in 2 patients (8%), complete blindness and visual acuity deficit in one patient each (4%). At the last visit, no change in visual function was detected in those with ophthalmological deficit and 5 (20%) of the patients with other neurologic manifestations still had residual neurological deficit. CONCLUSION: Arterial stroke in children is a rare event and only a few are referred for an eye examination. In view of the significant ophthalmological manifestations revealed in the current study, children with arterial stroke should be referred for early ophthalmological evaluation.