BACKGROUND: Posttransplantation lymphoproliferative disorder (PTLD) involving the central nervous system (CNS) is a rare and serious complication associated with solid organ transplantation. We treated a case of PTLD with CNS involvement in a liver transplant recipient and reviewed the literature. METHOD: The clinicopathological features of a 53-year-old man were retrospectively analyzed. RESULTS:Metastasis of the hepatoma was preoperatively considered on the basis of clinical ifndings. Craniotomy was performed and PTLD was diagnosed pathologically. The patient was treated with antiviral agents, radiation therapy, and chemotherapy; the immunosuppressive medication was reduced. The patient is still alive after follow-up for 14 months. CONCLUSIONS:Deifnitive diagnosis of PTLD is only established on the basis of histopathologic evaluation of the tissue. Although there are several ways to manage PTLD with CNS involvement, the prognosis is still poor.