Carotid web (CA-Web) is defined as a rare form of focal intimal fibromuscular dysplasia, which is protruded into the lumen and forms a membrane-like shelf in the posterior aspect of the internal carotid artery (ICA) bulb. It could lead to flow stagnation and thrombus formation in the proximal ICA, and has been increasingly recognized as a potential source of thromboembolism in young patients with recurrent cerebral ischemic events in the anterior circulation of an undetermined cause. In the general population, the prevalence of CA-Web is unknown and it is rarely found among stroke patients. However, CA-Webs are more frequent among young patients with cryptogenic ischemic stroke. Despite antiplatelet therapies, these patients appear to have a higher risk of recurrent ischemic stroke. As alternatives, anticoagulation, surgical resection, and carotid artery stenting are being considered, but largely based on anecdotal evidence.