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Carotid web (CW) is a non-atheromatous and non-inflammatory linear filling defect which is an under-recognised aetiology of embolic stroke of undetermined source, particularly in the younger population under 60 years of age. Due to limited epidemiological data, imaging techniques and clinical experiences, CW may be underdiagnosed or misdiagnosed as other vascular pathologies such as atherosclerotic plaque. There are no established diagnostic or management guidelines for treating CW and preventing secondary stroke.We present the case of a male in his late 40s who experienced an ischaemic stroke due to occlusion of the M1 segment of the right middle cerebral artery: ipsilateral to the CW, which was subsequently managed surgically with a carotid endarterectomy. We review the epidemiology, pathophysiology, diagnostic tools and treatment options for this rare condition, and propose directions for future research to improve outcomes for patients with CW.