Background:Kaposi sarcoma (KS) is a neoplasm of the endothelial cells. It often manifests with multiple vascular nodules on the skin and other organs. It is a systemic, malignant and multifactorial disease and has a variable course. There are four types: classic, endemic, iatrogenic and HIV-associated. The primary presentation on the penis and face is uncommon and is mainly observed in HIV-positive patients. Multiple treatment modalities are used including surgery, cryotherapy, electrosurgery, laser and radiation therapy.Main observation:The authors present two cases of isolated Kaposi sarcoma in HIV negative, human herpes virus 8 (HHV-8) positive non immunocompromised patients. One case with facial KS and the other one with penile KS. Both were treated surgically with no recurrence in the following 6 months of the follow up period.Conclusions:Kaposi sarcoma is rare in HIV negative patients and is associated with HHV-8 infection. Lesions are usually solitary and can be treated surgically. It should be included in the differential diagnoses of penile and facial lesions that are clinically suspecious and resistent to therapy.