Central retinal artery occlusion (CRAO) is a devastating ocular emergency characterized by acute painless visual loss in the ipsilateral eye. We describe the case of acute non-arteritic CRAO associated fusiform internal carotid-ophthalmic artery aneurysm with intraluminal thrombus. Despite the rarity of this condition, we suggest that carotid-ophthalmic artery aneurysm should be included in the differential diagnosis of CRAO.