Primary Sjogrens syndrome (pSS) is a systemic as well as an organ-specific autoimmune disease characterized by lymphocytic infiltration of the glandular epithelial tissue. It has been reported that pSS patients have a relatively increased risk for the development of lymphoma and various factors such as cytokine stimulation, environmental exposures and viral infections as well as genetic events may contribute to the development of lymphoma in pSS patients. Over the past few decades, numerous efforts have been undertaken to search for the relationship between lymphoma and pSS, for example advances in molecular biology for clonality analysis and well-linked register cohort studies for the predictive value of clinical, laboratory and histological findings. Despite this, mechanisms and prediction of lymphoma development in pSS patients still remain to be defined. In this review we have summarized the current knowledge concerning incidence and risk factors of lymphoma development in pSS patients. In addition, the most recent discoveries in the emergence and treatment of lymphoma in pSS patients and the possible mechanism of lymphoma development are also discussed.