Abstract: Kikuchi-Fujimoto disease (KFD) is characterized by lymphadenopathy and fevers. Skin lesions are seen in about 40% of the patients. The lymph node biopsy specimen shows coagulative necrosis with abundant karyorrhectic debris. KFD is often misdiagnosed as tuberculosis, systemic lupus erythema and hematological malignancies. The symptoms of the disease usually resolve spontaneously in 1~ 4 months with a low recurrence rate. Only symptomatic treatment is needed and definitive diagnosis to avoid excessive treatment is necessary. The pathogenesis, clinical manifestations, diagnosis and treatment of KFD are reviewed in this article.