Central retinal artery occlusion (CRAO) is a rare condition. It is a diagnostic and therapeutic emergency. CRAO is analogous to an acute stroke of the eye. The disease usually affects patients after 60 years of age, and rarely young persons. The risk factors for a CRAO are similar to ischemic stroke. It is due to embolic, thrombotic, or coagulation disorders. Typically, patients with acute CRAO present monocular, painless, and severe loss of vision. We report a rare case of a young 33-year-old woman who presented an atypical ischemic stroke revealed by a unilateral CRAO. This case reports a rare condition affecting a young patient, and highlights the interest of ophthalmologic examination in the diagnostic of a neuro disease and the early management of ischemic stroke.