Carotid artery stenting (CAS) is a reasonable alternative to carotid endarterectomy (CEA), especially in patients at high risk for surgery. Carotid artery thrombosis of the treated segment is a rare, early but potentially devastating complication of this endovascular procedure. The aim of this article is to identify and critically review cases of acute stent thrombosis reported in the literature.Previous trials that compared CEA with CAS were rather heterogeneous and not large enough to allow reliable conclusions. Furthermore, because there is limited follow-up information to date, the long-term effect of CAS remains unclear. Acute carotid thrombosis after angioplasty and stenting is a very rare but potentially fatal complication, and there are very few reports in the literature. This article reviews twelve cases of acute carotid thrombosis published in the English literature from eight different Vascular and Radiology Departments around the world.The different ways of immediate treatment of this rare complication of acute carotid thrombosis after CAS are, open surgical procedure with thrombus removal and thromboendarterectomy with or without removing of the stent, selective local or facilitated thrombolysis with the rescue use of GPIs (glycoprotein IIb/IIIa receptor inhibitors), recanalization by instent percutaneous transluminal angioplasty with distal protection and additional stent placement on the stented portion of the internal carotid artery (ICA) in conjunction with the intravenous administration of recombinant tissue plasminogen activator (rtPA: 1300,000 IU).Carotid artery stenting has to be performed under specific pro- and post procedure protocol from experienced endovascular specialists. The treatment of acute carotid thrombosis after CAS must be urgent and immediate in order to regain restoration of blood flow and avoid major neurological adverse events.