Up to 9% of patients with stroke or transient ischemic attacks (TIA) studied in hospital-based series will have a symptomatic internal carotid artery (ICA) occlusion. Common symptoms of carotid territory ischemia are motor and sensory involvement of the contralateral extremities, isolated motor dysfunction, isolated sensory loss, and least commonly, isolated dysphasia. Rarely, patients with bilateral ICA occlusions or severe stenosis may present with attacks that mimic vertebrobasilar territory ischemia. Chronic ischemia to the retina leading to chronic low perfusion can result in widespread retinal ischemia. A number of promising therapies exist for patients with carotid artery occlusion. Careful risk stratification according to presenting symptoms, cerebral metabolism, and cerebrovascular hemodynamic studies is essential to provide optimal management and identify potential subsets of patients that may benefit from external carotid to internal carotid (EC-IC) bypass surgery, contralateral carotid endarterectomy, or other interventions.