A 34-year-old woman presented with a 10-year history of recurrent oral and genital ulcerations and recurrent episodes of bilateral conjunctivitis associated with HIV infection. A diagnosis of Behçet's disease (BD) in association with keratoconjunctivitis sicca (KCS) was made after exclusion of other viral and autoimmune diseases according to the international criteria for BD. This is the first reported case of a combination of BD and KCS in a patient with HIV infection in which a complete resolution was observed as a result of successful highly active antiretroviral therapy. The likelihood that a direct viral effect or HIV-induced autoimmune mechanisms act in the pathogenesis of both BD and KCS in HIV-infected patients is discussed.