Posttransplantation lymphoproliferative disorders include a wide spectrum of diseases ranging from hyperplastic-appearing lesions to frank non-Hodgkin lymphoma. More than 90% of these disorders are Epstein-Barr virus-associated lesions of B-cell origin that arise in the setting of pharmacologic immunosuppression after transplantation. With the increased use of organ transplantation and intensive immunosuppression, posttransplantation lymphoproliferative disorders are becoming more common. The prognosis is often poor, with most patients dying despite receiving treatment. The aim of this review is to report the most recent knowledge about the clinical features, diagnosis, prophylaxis, and treatment of posttransplantation lymphoproliferative disorders, which can be useful to physicians and health assistants dealing with these life-threatening, posttransplantation clinical entities in clinical practice.