Over the last decade, it has become clear that iatrogenic immunodeficiency-related lymphoproliferative disorders can occur in non-transplantation settings. These lymphoproliferative disorders occur predominantly in patients with rheumatologic diseases who are treated with immunomodulatory drugs. Like immunodeficiency-related lymphoproliferations in other settings, these represent a spectrum of lymphoid neoplasms and are frequently associated with Epstein-Barr virus. The distribution of histologic types of iatrogenic lymphoproliferations in non-transplantation settings appears to differ from that seen in other immunodeficiency settings with a probable increase in representation of Hodgkin's disease and lymphoproliferations resembling Hodgkin's disease. Recognition of these immunodeficiency-related lymphoproliferative disorders is important for appropriate patient management.