Objective: To review the clinical features and outcome of all reported cases of Kaposi's sarcoma in patients with rheumatic diseases. Methods: In addition to our patient, we identified cases from a Medline search between the years 1966 and 2002. Cases associated with human immunodeficiency virus infection were excluded. Results: Including our patient, there were a total of 25 cases reported (11 men and 14 women). Rheumatoid arthritis was present in 8 cases, polymyositis/dermatomyositis in 5, vasculitis syndromes in 5, systemic lupus erythematosus in 3, polymyalgia rheumatica in 2, and 1 each of undifferentiated connective tissue disease and Behcet disease. All but 1 patient had been given systemic corticosteroids for a duration that ranged from 6 weeks to 22 years, and immunosuppressive drugs from 25 days to 3.5 years. The Kaposi's lesions usually involved the skin on the extremities; internal organ involvement occurred in 7 cases. Most lesions responded to a decreasing dosage of corticosteroids and immunosuppressive drugs, or to the administration of radiation or cytotoxic therapy. Six patients died, 4 of which were related to the progression of Kaposi's sarcoma. Conclusion: Kaposi's sarcoma in patients with rheumatologic conditions is rare. The clinical features are similar to those with classical Kaposi's sarcoma. Tumor regression usually occurs with decreasing corticosteroids and/or immunosuppressive drugs, local irradiation, or cytotoxic therapy. Semin Arthritis Rheum 32:326-333. Copyright 2003, Elsevier Inc. All rights reserved.