Epidemic Kaposi sarcoma (KS) is a vascular neoplasia with in situ malignancy, that serves as an AIDS defining illness and is associated with human herpes virus-8 (HHV-8). It has a multifocal nature, histologically presenting as a double proliferation of spindle cells and vascular spaces, probably of endothelial origin. AIDS-related Kaposi’s sarcoma is the most common malignancy in HIV-infected patients and is the most aggressive clinically form of the disease. The current researchers regarding the epidemiology and pathogenesis of Kaposi’s sarcoma aim to highlight therapeutic opportunities in both the developed and developing world.