A 35-year-old woman had been suffering for 10 years from a dry mouth and dry eyes without being medically examined. When she finally saw a doctor in September 1996, a chest radiograph revealed bilateral hilar lymphadenopathy. Histopathological examination of the lung and scalene lymph nodes revealed non-specific lymphadenitis. She was followed thereafter without any therapy. In 1997, she was referred to our hospital because of a high titer of antinuclear antibody. A diagnosis of Sjögren's syndrome was made on the basis of the results of sialography, lip biopsy, Schirmer's test, and the present of anti-SS-A antibody. Re-evaluation of the mediastinal lymph nodes and the lung by thoracoscopic biopsy revealed non-caseating epithelioid cell granulomas, which led to a diagnosis of sarcoidosis. Although the coexistence of Sjögren's syndrome and sarcoidosis has been reported occasionally, cases with histological proof of sarcoidosis have been rare. In a survey of 27 reported cases, the majority of the patients were female and in roentgenological stage I. In most cases, Sjögren's syndrome preceded sarcoidosis. The predominance of the Th 1 immune response at the site of each disease may be involved in the pathogenic mechanism by which these diseases coincide.