Kaposi sarcoma (KS) is an angioproliferative mesenchymal neoplasm caused by Kaposi sarcoma-related herpesvirus. This review outlines our current understanding of the epidemiology, pathogenesis, clinical presentation, and staging for this disease. Recent research has informed a more comprehensive understanding of the epidemiology of KS in the post-antiretroviral therapy era, and highlights the continued need to better characterize the African endemic subtype. Advances in clinical oncology, including checkpoint inhibitors and new skin-directed therapies, have translated into exciting new developments for the future of KS treatment options.