Kaposi sarcoma (KS) is an angioproliferative disease associated with human herpesvirus 8 (HHV-8) infection. Four clinical variants have been described, all unified by common histopathologic features. We report a case of KS in an elderly Italian man with an unusual clinical presentation characterized by a relatively rapid onset of lesions and an unexplained low CD4 lymphocyte count. We review KS, including the 4 recognized variants, the central histopathologic findings, and various treatment options. In addition, we briefly discuss causes of CD4 lymphocytopenia, including a cursory overview of idiopathic CD4 lymphocytopenia (ICL).