Systemic lupus erythematosus is a severe cutaneous-systemic disorder of unknown etiology. It is represented with erythematous patches on the face in 1 so-called butterfly distribution, and characteristically classified as an autoimmune disease with antinuclear antibodies. The autoimmune diseases such as systemic lupus erythematosus, Sjogren syndrome, rheumatoid arthritis have been associated with lymphoid malignancy-leukemia, malignant lymphoma-which could involve various organs(spleen, liver, brain, mediastinal lymph node, supraclavicular lymph nod, inguinal lymph node, cervical lymph node etc.). Many authors have studied about the association of systemic lupus erythematosus and malignant lymphoma, but exact etiology is still unknown. A common viral etiology for systemic lupus erythematosus has been suggested since virus-like particles have been found in the glomerular endothelium of patients with systemic lupus erythematosus. These oncogenic viruses may be responsible for the higher frequency of malignant lymphoma in patients with systemic lupus erythematosus. In the other theory, the causes of malignant lymphoma are the defect of immune system due to systemic lupus erythematosus and the long-term use of therapeutics for treatment of systemic lupus erythematosus. When the cellular immune system(delayed hypersensitivity) is impaired by immunosuppressive drugs, it is likely that the body is no longer able to recognize and reject malignant cells as they arise; they continue to grow and divide unhindered. The impairment of the cellular immuno system may allow growth of oncogenic virus or the survival of neoplatic tissue. 47-year old female patient treated systemic lupus erythematosus with steroid and immunosuppressive drugs for 5 years visited to our hospital due to elevated mass on left upper anterior maxilla area. By performing biopsy, we diagnosed this lesion as malignant lymphoma and referred to oncologist for chemotherapy. So we report a case of malignant lymphoma due to systemic lupus erythematosus with review of literatures.