BACKGROUND Acute carotid stent thrombosis (ACST) is a rare but devastating complication in the carotid artery stenting (CAS) procedure. The aim of this article is to report a case and review cases of ACST reported in the literature, and investigate risk factors and management strategies for ACST. CASE SUMMARY We reviewed the treatment process of a patient with ACST after CAS. Then multiple databases were systematically searched to identify studies reporting ACST from 2005 to 2020. The demographic data, risk factors, treatment strategies, and prognosis were extracted and analyzed. CONCLUSION The reason for ACST is multifactorial. Proper patient selection, normative anti-platelet treatment, and perfect technical detail may decrease the incidence of ACST. Several treatment strategies such as thrombolysis, mechanical thrombectomy, and open surgery may be options for the treatment of ACST. Limited data have shown that carotid endarterectomy is effective with favorable results.