• We treated four children with clinical symptoms and laboratory findings suggestive of Sjögren's syndrome (SS). We also review the findings in 23 children with the diagnosis of SS whose cases were reported in the literature. We propose that the following criteria for the diagnosis of SS, which are mostly used in adults, should also be applied to children: (1) keratoconjunctivitis evidenced by a Schirmer test and a quantitative rose bengal test; (2) xerostomia shown by a decreased basal and stimulated salivary flow; (3) lymphocytic infiltration in a minor salivary gland biopsy specimen with at least two foci per 4 mm2; (4) laboratory evidence of a systemic autoimmune disorder on the basis of a rheumatoid factor of 1/160 or greater, antinuclear antibody of 1/160 or greater, or extractable nuclear antigen antibodies. Only close observation and long-term follow-up of these patients will allow a better insight in the natural history of SS in children. Those children who do not fulfill these diagnostic criteria also need close and prolonged follow-up study: one of the possibilities is that their conditions will ultimately evolve toward definite SS.