A 70 year-old female with a 6-year history of primary Sjögren syndrome was admitted with fever and loss of weight. Examinations such as the lip biopsy reconfirmed the diagnosis of primary Sjögren syndrome. Abdominal CT and gallium scan showed lymphadenopathy in the abdominal cavity, and an exploratory biopsy was done. Lymph nodes and liver biopsy specimens revealed multiple noncaseating granulomata with some Langhans' giant cells, consistent with sarcoidosis. It has been previously proposed that connective tissue disease and sarcoidosis are related in terms of pathogenesis because both diseases share common immunological features and abnormalities in regard to cellular and humoral immunity. Indeed some data in the literature show a striking similarity between the lymphocyte subpopulation in the parotid gland of Sjögren syndrome and the bronchoalveolar lavage fluid of sarcoidosis. However, it is difficult to assume the same pathogenesis for both diseases, because this aspect of the diseases has not been completely clarified. Despite the fact that the two share common immunological features and abnormalities, the coexistence of primary Sjögren syndrome and sarcoidosis has rarely reported.