Takayasu`s arteritis (TA) is a chronic inflammatory disease which involves large vessels such as aorta, its major branches, and pulmonary vessels, and develops into an obstructive process. The disease affects most frequently oriental races, especially young women, and its etiology has not been found. Systemic lupus erythematosus is a disease which involves mainly small vessels and has been unusually reported to accompany TA in foreign reports, but not yet in Korea. The authors observed a rare case in which a patient diagnosed to have TA at the age of 30, and was accompanied by lupus nephritis. This case doesn`t have its precedent in Korea and we review of literatures and report the case.