In this review, we focus on the current understanding of the diagnosis of human herpesvirus 8 (HHV8)-associated lymphoproliferative disorders-a group of entities that range from hyperplastic proliferations to frank lymphomas. These diseases tend to occur in immunodeficient patients, but may occur in immunocompetent individuals as well. In recent years, we have learned of occasional cases with overlapping features among HHV8 entities, such as lesions intermediate between primary effusion lymphoma and HHV8-positive diffuse large B-cell lymphoma, not otherwise specified or cases sharing features of multicentric Castleman disease and germinotropic lymphoproliferative disorder. There is also a significant clinical overlap between these entities. It is important to have a better understanding of the biology of these lesions and to refine diagnostic criteria of these lesions, as the use of immunosuppressive agents to treat a variety of diseases, the expanded use of transplant as a therapeutic modality for a variety of cancers and organ failure patients, and the extended longevity of HIV-positive patients will likely result in an increased incidence of these lymphoproliferative processes in the future.