<h4>Background</h4>Kaposi sarcoma herpes virus (KSHV) is associated with multiple clinical manifestations, including primary effusion lymphoma, an aggressive CD38+ B cell lymphoma with a plasmablastic phenotype.<h4>Case</h4>This case describes an antiretroviral therapy-adherent person with HIV who presented with concurrent KSHV-related disorders of Kaposi sarcoma (KS), multicentric Castleman disease (MCD), and extracavitary primary effusion lymphoma (EC-PEL). This evidence concerns the gene CD38 and primary effusion lymphoma.