Kaposi's sarcoma (KS) is an unusual form of tumor which in the era of HIV/AIDS pandemic is increasingly observed outside the original endemic areas. It was shown that the development of KS is in directly related to infection with human herpes virus 8 (HHV-8). The pathophysiology of KS is complex and is influenced by HIV co-infection and by global cytokine interactions. Skin, gastrointestinal tract and respiratory organs are typically involved. A good therapeutic effect of combined antiretroviral therapy (cART) was documented. We provode a review of the current knowledge of the pathophysiology of and therapeutic options for KS and one clinical case.