Posttransplant lymphoproliferative disorder is a heterogenous group of clonal hyperplasia/neoplasms that can range from benign to highly malignant lesions. Mortality rates can approach 60%. This entity has been on the rise for the last 2 decades with the advent of highly potent immunosuppressive agents. Epstein-Barr virus has shown to play a primary role in more than 90% of the cases. Although standard protocols do not exist for primary prevention and treatment, new agents are emerging that may play a role in treatment and prevention of this debilitating, and at times, fatal disease.