The Epstein-Barr virus is a ubiquitous human herpesvirus that is associated with an increasing number of human malignancies. Among these are Epstein-Barr virus-associated lymphoproliferative diseases in immunocompromised patients, a spectrum of mainly B-cell diseases that range from polyclonal lymphoproliferative diseases, which resolve when immunosuppression is halted, to highly malignant lymphomas. Progress has identified Epstein-Barr virus gene products involved in B-cell transformation, variation in Epstein-Barr virus transforming genes, distinct target cell populations with differing regulation of Epstein-Barr virus expression, and selective recruitment of other supportive cell types as factors in the heterogeneity of lymphoproliferative diseases. New therapeutic approaches to treat lymphoproliferative diseases are also being developed. Finally, xenotransplantation poses new risks for the introduction of Epstein-Barr virus-like viruses and more aggressive lymphoproliferative diseases in heavily immunosuppressed patients.