The aim of this meta-analysis was to determine the risk of lymphoma development for several types of autoimmune disorders, such as primary Sjogren syndrome (SS), systemic lupus erythematosus (SLE), and rheumatoid arthritis (RA). In total, the studies included 8700 patients with SLE, 95 104 with RA, and 1300 with SS. The meta-analysis showed that lymphoma is more common in patients with autoimmune diseases than in the general population. There is a high risk of lymphoma development in SS, a lower risk in SLE, and even a lower risk in patients with RA. In patients with RA, the use of cytotoxic or immunomodulatory agents may increase the risk of lymphoma. In this setting, the association of lymphoma with autoimmune diseases generates interest because it may enlighten the mechanisms of lymphomagenesis and provide new tools for therapy.