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Ocular ischaemia due to a spontaneous carotid artery dissection. Internal carotid artery dissection (ICAD) is caused by the disruption of the tunica intima, with the formation of an intramural haematoma that can cause stenosis or occlusion of the artery's lumen, leading to reduced blood flow and secondary thrombus formation. Up to two-thirds of patients with ICAD show ophthalmological symptoms or signs, which are, frequently, the first manifestations of this clinical condition, often preceding for weeks the neurological signs of cerebral infarction. Central retinal artery occlusion (CRAO) is a rare complication of ICAD, secondary either to haemodynamic compromise, with ocular hypoperfusion and reverse flow within the ophthalmic artery, or to thromboembolic events, in rarer cases. We report a case of CRAO secondary to a spontaneous ICAD, in an otherwise healthy middle-aged patient.