A 54-year-old female with Epstein-Barr virus (EBV)-associated B-cell lymphoma of the brain and Evans syndrome is presented. After treatment of the lymphoma with irradiation to the brain and chemotherapy she developed Evans syndrome with autoimmune hemolytic anemia and thrombocytopenia. Further immunosuppressive treatment for Evans syndrome caused the dissemination of EBV-associated B-cell lymphoma. The dissemination of EBV-associated B-cell lymphoma was confirmed by in situ hybridization with EBV encoded small RNAs (EBER), polymerase chain reaction with Bam HI-W fragment of EBV and lymphocyte determined membrane antigen (LYDMA) and immunohistochemistry with latent membrane protein (LMP). Since only a few cases of lymphomas associated with EBV occurring during autoimmune diseases have been reported, this is an illustrative case.