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Background: Carotid web (CaW) is a rare disease, but it is associated with cryptogenic recurrent ischemic stroke. Due to a lack of epidemiological data and a generally low awareness of the disease, CaW is difficult to diagnose and is even prone to misdiagnosis. It is often treated with medical management, carotid endarterectomy (CEA) and stent implantation. These therapies are aimed at preventing cerebrovascular events. Case presentation: We describe the case of a young adult patient who suffered ischemic stroke for unknown reasons. He was successfully treated with CEA and finally diagnosed with CaW. Recommendation: People should improve their knowledge of CaW. The correct diagnosis of CaW is important for its therapeutic strategies. Medical management alone may not effectively prevent recurrent stroke for patients with CaW. CEA is an ideal option to treat CaW for lesion removal, obtaining the pathologic results and engaging in satisfactory short-term follow-up.