BACKGROUND Posttransplant lymphoproliferative disorder (PTLD) is increasingly recognized as a serious complication of solid organ transplantation in both children and adults. Factors associated with increased risk of PTLD include mismatch of recipient and donor EBV serologic status (seronegative recipient with seropositive donor), and intensive drug-induced immunosuppression. RESULTS We searched MEDLINE for articles published since 1970 to January 2009. Search terms included posttransplant lymphoproliferative disorder, immunosuppression, posttransplant malignancy, treatment, antiviral agents, rituximab, interferon alpha, chemotherapy, radiation, surgery. Studies in English of adult and pediatric populations after solid organ transplantation were selected and analyzed. CONCLUSIONS Screening of patients at risk and balancing the intensity of immunosuppression against the risk of allograft rejection could reduce the risk of developing PTLD. In patients who develop PTLD, the severity and extent of disease should be examined and an individualized treatment plan including immunosuppression reduction and other agents should accordingly be chosen.