Kaposi’s sarcoma (KS) has been originally described by Kaposi as an idiopathic multiple neoplasm of the skin composed of proliferating vessels and spindle-shaped cells. Its origin has been an issue of debate for long; there is evidence to believe that the tumor is derived from endothelial cells, particularly from lymphatic endothelium. There are four types of KS known today, based on their occurrence and the accompanying clinicopathological data. The classic KS has been first seen and described by Moritz Kaposi in Europe in 1872, an endemic type was recognized in 1960s in sub-Saharan Africa, a KS variant associated with immunosuppressive therapies (iatrogenic) has been reported by various groups in Western countries particularly in organ transplant patients, and the HIV-associated KS type, also called epidemic, first appeared in the United States in 1981. Human herpesvirus 8 (HHV-8) is considered as an etiological agent for all variants. Primary effusion lymphoma and multicentric Castleman’s disease are also linked with this virus.