Patients with carotid artery disease may present with monocular, ipsilateral ocular symptoms, and signs that can herald a devastating stroke. Asymptomatic retinal emboli, transient monocular visual loss, and central retinal artery occlusion are the most common. Venous stasis retinopathy and ocular ischemic syndrome are associated with severe hypoperfusion of the eye and usually reflect severe carotid occlusive disease. Embolic ischemic optic neuropathies and compression of the intracranial optic nerve by supraclinoid carotid arteries remain debated. Third-order Horner's syndrome and eye pain are common in carotid artery disease, particularly carotid dissection; carotid artery disease, however, is an uncommon cause of ocular motor nerve palsy. Several recent publications have highlighted the prognosis and management of patients with carotid artery occlusive disease, and have emphasized the role of the ophthalmologist in early diagnosis and prevention of further cerebral and systemic complications.