Sjögren’s syndrome is a chronic autoimmune exocrinopathy mostly involving salivary and lachrymal glands. Moreover, the incidence of lymphomas in primary Sjögren’s syndrome patients seems to be significantly elevated as compared to the general population. About 4-8% of patients with Sjögren’s syndrome develop non-Hodgkin’s lymphomas within the initial 10 years of the disease. Usually they are located in the major salivary glands. The majority of the lymphomas are of mucosa-associated lymphoid tissue (MALT) type, and usually have indolent course, with a 90% rate of five-year survival. Neoplastic transformation may be present in different organs not initially involved by lymphoproliferative lesions (the parotid and lachrymal glands). Although recent years have brought new information about malignant transformation, the process by which patients develop lymphomas still remains unclear. This article presents the clinical picture, risk factors and symptoms indicating evolution of immunoproliferative lesions into lymphoma in Sjögren’s syndrome.