Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is seen usually in patients with cervical lymphadenopathy and high fever. It may frequently occur in young women which has self limited process. KFD has a similar clinical and histopathological diagnosis and it is rarely accompanied with systemic lupus erythematosus (SLE). In this case report, a 36 years old woman who had axillary and cervical lymphadenopathy with fever, lose weight, arthralgia and skin rashes on her face has been admitted to hospital, is presented. Preliminary clinical diagnosis was KFD. Biopsy of enlarged lymph node showed SLE. We present this case because of the difficulties in differential diagnosing with SLE versus KFD. ©2007, Firat University, Medical Faculty