A 42-year-old woman presented in 1992 with Raynaud's phenomenon, polyarthralgia and morning stiffness and next year developed xerostomia and xerophthalmia. Investigations revealed 20 mm/hr of ESR, positive test for RAPA (1 : 320), ANF (1 : 1280), anti-SS-A antibody and anti-Wa antibody, and positive Schirmer tear test. A biopsied specimen of minor salivary gland of lip showed lymphocytic infiltration around the ducts and fibrotic changes and a sialogram demonstrated diffuse dilatations of the peripheral ducts. Resulting from the data shown above, the diagnosis of Sjögren's syndrome was made. Anti-Wa antibody, which recognizes a 48 kD tRNA associated protein was identified in serum from a patient with systemic sclerosis by Yamagata in 1985. So far the antibody has been specific for systemic sclerosis, because all of six patients with anti-Wa antibody previously reported were diagnosed as having systemic sclerosis. However, we here described the first case of Sjögren's syndrome with anti-Wa antibody.