Sjogren's syndrome (SS) is a systemic autoimmune disease that primarily affects the exocrine glands, resulting in dryness of the eyes and mouth due to lymphocytic infiltration of the salivary and lacrimal glands along with arthritis, kidney, liver, and lung involvement, chronic fatigue, musculoskeletal pain, vasculitis, and so on. Considerable advance has been made for the classification and treatment of primary SS in the past few years. This article reviews the recent classification criteria for primary SS and briefly discusses the conventional and novel therapies of the disease.