Carotid artery stenting (CAS) is an alternative treatment to carotid endarterectomy for carotid artery stenosis. Acute stent thrombosis (ACST) is an extremely rare complication but can have devastating consequences. Although many cases have been reported, the best treatment is still uncertain. In this study, we report the treatment of ACST caused by diarrhea in an intermediate clopidogrel metabolizer. We also review the literature and discuss appropriate treatment strategies for this rare event.