Carotid Artery Stenting (CAS) is an alternative strategy to prevent ischemic stroke in patients who are at high-risk of surgery compared with carotid endarterectomy (CEA) .Acute carotid stent thrombosis (ACST) was extremely rare, but devastating complication after CAS. Academically, it occurred within 30 days after CAS. There are several reasons causing ACST, such as inadequate antiplatelet therapy, early discontinuation of antiplatelet therapy, clopidogrel resistance, hypercoagulable state, local vessel dissection, vasospasm, intimal injury, and so on. Although successful recanalization cases were reported, however, there is still a lack of experience in the choice of treatment methods and the timing of ACST treatment, especially when the patient has clopidogrel resistance. We are here to provide a case that successfully revascularization after ACST in patient with evidenced clopidogrel resistance, which was further confirmed by genetic testing. In our case, both thrombus aspiration and platelet glycoprotein IIb/IIIa antagonist (GPIs) were used for recanalization. Also, we review the literature and discuss appropriate treatment strategies for this deadly and rare event.