We reviewed the recent findings in diagnosis, pathogenesis and management of Sjögren's syndrome. New diagnostic classification criteria for Sjögren's syndrome were published by the American College of Rheumatology in 2012, and validation was completed. Salivary gland ultrasonography has been examined as a diagnostic and prognostic marker for Sjögren's syndrome, and the results have revealed that it has high specificity and low sensitivity. Disease activity assessment tools for Sjögren's syndrome have been developed and validated for upcoming clinical trials. Several associated genes were identified by genome-wide association studies, with large cohorts in Europe and China. Several single nucleotide polymorphisms of Sjögren's syndrome are related to germinal center formation and lymphoma development. New treatment modalities, including interleukin-1 receptor antagonist, anti-CD20, anti-BAFF/Blys, and CTLA4-immunoglobulin were investigated, but the optimal therapeutic compounds have yet to be found.