Kikuchi-Fujimoto Disease (KFD), is a rare and self-limited condition of histiocytic necrotizing lymphadenitis, which typically presents as fever and lymphadenopathy. We describe a case of KFD in an Iranian woman. Due to low incidence and high importance, awareness of this disease is necessary for clinicians for early diagnosis and appropriate treatment. A 26-year-old woman was admitted to our hospital with a 3-week history of fever and lymphadenopathy. On physical examination, she had three separate enlarged lymph nodes on the right side of her neck. In laboratory tests that were carried out, she had mild anemia and an increase in C-reactive protein (CRP) level, erythrocyte sedimentation rate (ESR) and lactate dehydrogenase (LDH) level, while other tests were normal. Ultasound (U/S) guided core needle lymph node biopsy was performed and based on the histological finding, diagnosis of Kikuchi-Fujimoto disease was made. The patient was managed supportively and with prednisolone. She symptomatically improved and was discharged with no follow up. Although the incidence of KFD is rare, it must be considered as a differential diagnosis of lymphadenopathy especially in tuberculosis-endemic areas like our country-Iran. Moreover, it is necessary that physicians are aware of this disease in order to minimize unnecessary evaluation and toxic treatment.