ABSTRACT: Approximately 90% to 95% of Kaposi sarcoma cases occur in human immunodeficiency virus - infected homosexual and bisexual men. Pulmonary Kaposi sarcoma is uncommon in women, and rarely considered as a potential cause of diffuse lung disease in women with acquired immunodeficiency syndrome. The disease is usually mistaken clinically for pulmonary infection. A 32-year-old woman was admitted with a 2-month history of dyspnea, evening fever, hemoptysis, weight loss, and generalized adenomegaly. Physical examination showed erythematous macules in the lower limbs. Skin and open lung biopsy demonstrated Kaposi sarcoma. Computerized tomography demonstrated peribronchovascular interstitial thickening. Although uncommon, pulmonary Kaposi sarcoma should be considered in the differential diagnosis of diffuse lung disease in women with AIDS.