Primary Sj?gren’s syndrome is an autoimmune disease that mainly affects exocrine glands. Patients usually refer to ophthalmologists because of severe dry eye symptoms. Most clinicians have insufficient knowledge with dry eye disease associated with primary Sj?gren’s syndrome probably leading to misdiagnosis or missing the diagnosis.The diagnosis of Sj?gren’s syndrome dry eye disease (SS-DED) is difficult, but the extremely invasive objective examination and the development of biomarkers will help to understand this disease and explain its pathogenesis from a new perspective. There is no specific treatment for the SS-DED, and most patients should receive multiple treatments to select the optimal treatment.