During the last 15 years, KS has been elevated from a position of only limited academic interest to the distinction of being the most common malignancy seen in HIV-infected patients. Ophthalmologists need to become versed in the proper diagnosis and management of this condition, as ocular involvement may be seen in up to 1 in 5 patients with KS. The possibility of occult HIV disease should be entertained in a young person with an atypical hordeolum or subconjunctival hemorrhage, as KS sometimes mimics these common lesions and represents the initial presenting sign of AIDS. The patient with ocular lesions must also be evaluated appropriately for life-threatening visceral disease. Current concepts regarding the pathogenesis of KS center on a model in which an initial event, possibly infection by human herpesvirus 8, transforms normal mesenchymal cells such that they become abnormally sensitive to the high levels of cytokines present during HIV infection. Subsequent proliferation and additional mutational events result in clinically apparent disease. Present treatments include systemic chemotherapy for widespread disease and local methods such as excision, cryotherapy, radiotherapy, and intralesional injection. However, the majority of ocular lesions may be followed up with observation only. The appropriate strategy to pursue depends on the overall clinical scenario, including the patient's general health, the extent of disease, the degree of morbidity secondary to local ocular tumors, and the size of the lesions to be treated. Future therapeutic options will be aimed at modulating specific pathogenetic factors responsible for tumor development, including host cytokines, viral replication factors, and angiogenic mediators.