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Background Retinal artery occlusion can lead to sudden visual loss without pain. The acute management of retinal artery occlusion remains unresolved. Case Report A 65-year-old male was hospitalized to an emergency room for visual loss on the left side within 6 hours of onset. Combined occlusion at retinal artery and ciliary artery was confirmed by an ophthalmologist and we assessed ophthalmic artery occlusion. However, MRA revealed no significant steno-occlusion of internal carotid artery. Transfemoral cerebral angiography was carried out immediately and showed a movable thrombus at the orifice of the ophthalmic artery. We decided on endovascular thrombectomy to prevent permanent visual loss. Finally, his visual acuity was improved after successful thrombectomy. Conclusions Although MRA is intact, small thrombus right at the orifice of the ophthalmic artery can cause a sudden monocular visual loss due to occlusion of the retinal artery. In this setting, urgent endovascular thrombectomy can offer visual improvement.