OBJECTIVE： To investigate the clinical characteristics and gene polymorphism of oxcarbazepine （OXC）- induced Stevens-Johnson syndrome （SJS） and toxic epidermal necrolysis （TEN）. METHODS： Retrieved from CNKI， Wanfang， VIP， PubMed， EMBase， SpringerLink and other databases， case reports about OXC-induced severe ADR were summarized and analyzed. RESULTS： Twelve literatures were collected， and 13 case reports about OXC-induced SJS/TEN were obtained. Male had more OXC-induced severe skin ADR than female. ADR mostly occurred during 1-14 d after medication. All patients were cured with treatment of glucocorticoid and antiallergy， without death case. Genotyping for 8 patients were performed and 6 of them showed the presence of HLA-B*1502 allele. While HLA-B alleles of 2 patients were HLA-B*1518/B*4001， which was the variation of HLA-B*1502. CONCLUSIONS： OXC-induced ADR should be monitored closely. Great importance should be attached to patient education and follow-up program. HLA-B*1502 gene detection should be performed to guide rational use of OXC and optimize clinical drug use plan.