Kaposi’s sarcoma (KS) was first described 100 years ago. This tumor of endothelial origin starts in the skin or mucous membranes. The classical form is rare, affects elderly persons of Mediterannean origin and is generally indolent. The African variant develops in children and young men, showing an aggressive and usually fatal course. The third variant of KS affects immunodeficient patients, particularly renal transplant recipients and HIV-infected persons. The epidemic Kaposi’s sarcoma (EKS) is the most common tumor of HIV-infected patients with an incidence of up to 50%. The cause of EKS still remains to be determined. The clinical progression of the disease is closely related to the immunologic defect. This tumor generally appears with multiple skin, oral lesions and/or lymph node involvement. Progressive EKS may infiltrate lungs and the gastrointestinal tract. Treatment of EKS must take into account the extent of the tumor and debilitating symptoms or tumor-associated severe to life-threatening complications. Therapeutic approaches include local treatment with radiotherapy, intralesional injections of dilute chemotherapy, topical application of liquid nitrogen, laser treatment or systemic therapy with interferon or cytostatic agents. The choice of therapy should be based on the specific needs of the individual patient. EKS-associated clinical symptoms, immunological status and other HlV-in-duced diseases, particularly opportunistic infections, influence the therapeutic decis