Kikuchi’s disease called also Kikuchi- Fujimoto’s disease or Histiocytic necrotizing lymphadenitis is a rare entity. It is a self-limiting process of unknown etiology first reported in Japanese literature separately by Kikuchi and Fujimoto in 1972. Then similar cases have subsequently been reported throughout the world. We reported a case of Kikuchi's disease in a. 38-year-old man presenting with generalized lymphadenopathy and persistent fever throughout more than 2 months. It’s the first case was diagnosed in Al muwasat University Hospital in Syria. We reviewed the literature to summarize and report the most common clinical, laboratory and histology characteristics of Kikuchi- Fujimoto’s disease.