【Abstract】 Objective To study the relationship between autoimmune disease and malignant lymphoma. Methods The clinical data of a patient with autoimmune disease complicated with malignant lymphoma and the relevant literature were analyzed. Results The patient had a history of autoimmune hemolytic anemia for 9 years and had been treated with glucocorticoid for a long time. The EB virus was infected in the course of the disease. Finally, the marginal lymphoma was diagnosed by bone marrow immunophenotyping and bone marrow biopsy. Conclusion Autoimmune hemolytic anemia gradually evolved into the marginal area of lymphoma cases is rare. The pathogenesis may be associated with viral infection, immune dysfunction, immunosuppressant applications, immune surveillance out of control and so on.