The case of a 61 year old woman with Sjögren's syndrome with aortic and mitral stenosis is reported. She suffered from rheumatic fever at a young age. Physical and echocardiographic examinations showed findings of mitral and aortic valve stenosis. In addition, she had experienced xerostomia, a gritty sensation in the eyes and Raynaud's phenomenon. Blood examination showed hypergammaglobulinemia, positive rheumatoid factor, antinuclear and anti-Ro (SS-A) antibodies. The diagnosis of Sjögren's syndrome was confirmed by sialography and biopsy of the labial salivary gland. The combination of valvular disease and Sjögren's syndrome is rare and the etiological correlation is discussed.