Sickle cell trait (SCT) is traditionally considered a benign condition by ophthalmologists. Several studies have reported ocular complications in SCT, but these complications have been described as a consequence of trauma, exertion, and associated systemic disorders. We here in the report a case of an Arab teen boy, who presented with a sudden loss of vision in his left eye of 1 h duration. The ocular examination revealed acute central retinal artery occlusion. He underwent a series of laboratory and radiological investigations. The blood investigations revealed SCT and abnormal partial thromboplastin time. The fundus fluorescein angiography revealed abnormal retinal vascular perfusion. Marked blood rheological impairment and activation of the coagulation pathway can occur without any contributing factors in SCT leading to severe ocular complications. This is one of the young patients with spontaneous vascular occlusion in SCT.