Carotid webs (CaWs) are atypical forms of fibromuscular dysplasia characterized by a fibrous, shelf-like intimal flap projecting into the arterial lumen from the posterior wall of the internal carotid bulb. CaWs can disrupt normal blood flow, leading to stasis and thrombogenesis, thereby increasing the risk of downstream embolic strokes, particularly in young patients without other vascular comorbidities. Despite their clinical importance, there are no clear guidelines for managing CaWs. This review discusses the epidemiology, pathogenesis, pathophysiology, diagnosis, and treatment options for CaWs. CaWs are associated with younger age, African American race, and female sex. They are rare, with a prevalence of about 1% among non-stroke patients, but can be bilateral in approximately half of affected patients. The risk of stroke recurrence is high, with rates ranging from 4.4% at 1 month to 20% over 2 years. The pathogenesis of CaWs is debated, with some suggesting congenital defects and others proposing acquired lesions. CaWs are thought to alter blood flow dynamics, leading to thrombogenesis and increased stroke risk. Diagnostic tools such as computed tomographic angiography (CTA), magnetic resonance (MR) angiography, ultrasound, and catheter angiography are used to detect CaWs, with CTA being the most widely used. Optical coherence tomography (OCT) is emerging as a valuable tool for diagnosis and characterization. Treatment options include medical management with antiplatelet therapy, carotid stenting, and carotid endarterectomy (CEA). Medical management alone may not be sufficient to prevent stroke recurrence, and carotid interventions can be safe and effective treatments. Stenting and CEA have shown low rates of periprocedural complications and high rates of preventing recurrent strokes. However, the choice between stenting and CEA should be individualized based on patient-specific factors. Future research is needed to optimize treatment strategies and better understand the pathogenesis and management of CaWs.Carotid webs (CaWs) are atypical forms of fibromuscular dysplasia characterized by a fibrous, shelf-like intimal flap projecting into the arterial lumen from the posterior wall of the internal carotid bulb. CaWs can disrupt normal blood flow, leading to stasis and thrombogenesis, thereby increasing the risk of downstream embolic strokes, particularly in young patients without other vascular comorbidities. Despite their clinical importance, there are no clear guidelines for managing CaWs. This review discusses the epidemiology, pathogenesis, pathophysiology, diagnosis, and treatment options for CaWs. CaWs are associated with younger age, African American race, and female sex. They are rare, with a prevalence of about 1% among non-stroke patients, but can be bilateral in approximately half of affected patients. The risk of stroke recurrence is high, with rates ranging from 4.4% at 1 month to 20% over 2 years. The pathogenesis of CaWs is debated, with some suggesting congenital defects and others proposing acquired lesions. CaWs are thought to alter blood flow dynamics, leading to thrombogenesis and increased stroke risk. Diagnostic tools such as computed tomographic angiography (CTA), magnetic resonance (MR) angiography, ultrasound, and catheter angiography are used to detect CaWs, with CTA being the most widely used. Optical coherence tomography (OCT) is emerging as a valuable tool for diagnosis and characterization. Treatment options include medical management with antiplatelet therapy, carotid stenting, and carotid endarterectomy (CEA). Medical management alone may not be sufficient to prevent stroke recurrence, and carotid interventions can be safe and effective treatments. Stenting and CEA have shown low rates of periprocedural complications and high rates of preventing recurrent strokes. However, the choice between stenting and CEA should be individualized based on patient-specific factors. Future research is needed to optimize treatment strategies and better understand the pathogenesis and management of CaWs.