Objectives The carotid web is a compelling potential mechanism of cryptogenic stroke. It is easy for it to escape diagnosis or be misdiagnosed, since it rarely causes hemodynamic stenosis. Currently, there is a lack of consensus on the prevalence, etiology, imaging evaluation, and treatment strategy of carotid artery. We aimed to present the multimodal imaging of carotid web and comprehensively review the characteristics of carotid web. Methods A 38 year-old man with carotid web, who presented with acute left hemispheric syndrome, was not identified by computed tomography angiography and high-resolution magnetic resonance imaging in the initial report, but were identified in retrospect and then confirmed by digital subtraction angiography and histopathology. A literature review of carotid web was performed to identify prevalence, nature history, imaging tools, and optimal management of carotid web. Results 80 reports including 681 patients with carotid web were identified. The prevalence of symptomatic carotid web was 1.2% in patients with transient ischemic attack/stroke, 6.4% in patients with cryptogenic ischemic stroke, 1.1% in patients with large vessel occlusion stroke, and 4.4% in patients who operated on for carotid stenosis. A total of 23.0% patients had bilateral carotid web. In most patients, carotid web was located on the posterior wall (87.3%) of the carotid artery (98.7%). The mean length was 3.3 mm and mean stenosis rate was 20.9%. A total of 31.6% percent patients had thrombus trapped in carotid web and 75.9% patients had large vessel occlusion. Computed tomography angiography and digital subtraction angiography were favorable tools to detect carotid web. There were rare periprocedural complications and no recurrent strokes in carotid revascularization patients. Conclusions carotid web is an under-recognized cause of ischemic stroke. It is easy to be missed or misdiagnosed. Carotid revascularization can effectively prevent recurrent stroke for patients with symptomatic carotid web. Clinicians should strength their learning and understanding of carotid web.