Kaposi sarcoma (KS) is a mesenchymal tumour associated with human herpesvirus-8 (HHV-8) infection. However, the incidence of HHV-8 infection is far higher than the prevalence of KS, suggesting that viral infection per se is not sufficient for the development of aggressive phenotype and that one or more additional cofactors are required. The great geographical variation in African-endemic and classic KS incidence points to a role for environmental factors in the etiology of Kaposi sarcoma. However, there are few unequivocably established environmental factors involved in KS pathogenesis. This review focuses on the environmental factors thought to be associated with KS, more particularly iron exposure and facilitation of transmission of HHV-8 infection by contact with blood-sucking arthropods.