Primary Sjögren’s syndrome has been generally acknowledged as a chronic benign autoimmune disease. Patients with Sjögren’s syndrome have an increased risk of non-Hodgkin lymphoma compared with the general population, and this increased risk is evident for both primary and secondary forms of Sjögren’s syndrome. Persistent salivary gland enlargement, lymphadenopathy, Raynaud phenomenon, presence of antibodies to SS-A or SS-B, rheumatoid factor, and low serum complement C4 are risk factors of developing lymphoma. Non-Hodgkin lymphomas complicating Sjögren’s syndrome are characterized by an indolent course, small tumour burden, and an excellent performance status. Lymphomagenesis exemplifies the development of antigen-driven B-cell lymphoma and the control of disease activity is the primary objective of the management of the patient in order to repress chronic B cell stimulation.