Kaposi Sarcoma (KS) is most commonly associated with Acquired Immunodeficiency Syndrome (AIDS). Kaposi sarcoma herpesvirus is thought to play a huge role in the pathogenesis of KS. The diagnosis and management of KS can be quite challenging, and Physicians need high index of suspicion to diagnose KS as it can be mistaken for other skin pathologies. We present a case of a young male who developed KS on the penis as the initial manifestation of human immunodeficiency virus infection (HIV) and AIDS. Initially, he presented with a painful non-resolving ulcer on the glans penis for 2 weeks. He tested positive for HIV and his HIV viral load was more than 200,000 copies with CD4 count being only 8 per microliter. Histopathological examination of the lesion along with immunohistological staining were positive for KS. The patient was not adherent to his antiretroviral therapy (ART), and his condition deteriorated. Literature review showed only 16 cases of HIV positive patients presenting with KS involving the penile area, with only 4 of them being the initial manifestation of HIV and AIDS. A combination of systemic chemotherapy and ART is often needed for visceral or metastatic KS. There is a huge need to increase awareness about HIV and related complications among health care providers and the general population.