Carotid artery stenosis is a major risk factor for ischemic stroke. Although effective treatment options exist, careful assessment of benefits and risks for individual patients is needed in clinical decision-making. This article reviews contemporary treatments for carotid artery stenosis, the underlying evidence, and areas of uncertainties. Specific recommendations are available to guide the standard of care of carotid artery stenosis. Nonetheless, significant uncertainties are noted in patient selection for surgical treatment of asymptomatic carotid stenosis and in optimal treatment targets for pharmacological therapies. Advanced imaging has been used to predict future risk of ipsilateral stroke and clarify mechanisms of actions of pharmacological therapies, primarily in observational studies. Pharmacological and surgical treatments for extracranial carotid artery stenosis continue to evolve with many relevant clinical trials completed and clinical guidelines updated in recent years. Future clinical trials to tackle the areas of uncertainties are warranted.