Although carbamazepine (CBZ) is the most common cause of Stevens-Johnson syndrome (SJS), a new anticonvulsant, oxcarbazepine, which is structurally related to carbamazepine, has been shown to induce SJS, although extremely rarely. Recently, a strong association was found between human leukocyte antigen (HLA) B*1502 and CBZ-induced SJS/TEN in a Han Chinese population. Here, we report a case with SJS, which was induced by oxcarbazepine. HLA genotyping in the patient showed HLA-B*1518/B*4001. HLA-B*1518 is a HLA-B15 variant. The genetic significance of HLA-B*1518 in association with oxcarbazepine-induced SJS needs to be further studied.