Sjogren's syndrome (SS) is an autoimmune disease characterized by dryness of the mouth and the eyes. Systemic involvement in SS is well known, however, obvious cardiac manifestations, particularly significant valve disorders, are extremely rare and only three cases of significant valve disease associated with SS that required surgical intervention have been previously described. We report a case of aortic stenosis (AS) associated with SS in an elderly patient. The diagnosis of primary SS had been made based on clinical features, positive ocular signs, and positive serologic findings. Echocardiography showed severe calcification, elevated mean pressure gradient (57 mmHg), and a small orifice area (0.45 cm(2)) of the aortic valve. At surgery, severe calcification of the aortic cusps and the annulus was the mechanism of AS, and the aortic valve was replaced with a bioprosthetic valve. Valve pathology showed nodular calcification and hyaline degeneration, but lymphocyte infiltration was not evident. The etiologic relation of SS to the valve lesions is not clear pathologically in this case, however, chronic inflammation related to immunologic reactions in SS could have some effect on exacerbation for degeneration of the valve tissue.