Diseases of the head and neck often are the first sign of Human Immunodeficiency Virus (HIV) infection. As current treatment increases the survival time of HIV-positive patients, the physician may encounter patients with a greater variety and number of diseases related to the primary HIV infection. Some entities are harbingers of HIV infection, such as Kaposi's sarcoma and parotid cystic enlargement. Other manifestations, such as Mycobacterium avium complex infection, may be used to determine the stage of HIV infection. The advent of highly active anti-retroviral therapy (HAART) has transformed the prevalence of HIV-associated head and neck disease. This article reviews the clinical presentations and treatments of common and uncommon head and neck manifestations in HIV infection. Accurate assessment of these conditions may allow the physician to identify HIV infection in previously undiagnosed patients, and, as a result, provide more timely treatment.