Kikuchi-Fujimoto disease is a necrotizing lymphadenitis, involving young patients, predominantly females. Lymphadenopathy is usually localized, particularly in the cervical area, mostly unilateral and tender. Fever is present in one third of cases. Associated skin lesions, arthralgia, myalgia, splenomegaly or hepatomegaly are rare. Laboratory evaluation shows a slight increase of erythrocyte sedimentation rate and leukopenia. Kikuchi-Fujimoto has been reported in association with other diseases, including systemic lupus, Still's disease, hemophagocytosis, pregnancy, other autoimmune diseases, and cancer. A viral or bacterial origin has been suspected but not confirmed. Lymph node biopsy allows the diagnosis and shows necrotizing lymphadenitis with acidophil necrosis, CD68+ histiocyte infiltrate, presence of plasmacytoid monocytes, multiple apoptotic cells (CD8+ T cell) with nuclear dust, immunoblastic reaction and the absence of neutrophils or eosinophils. The disease course is usually spontaneously favourable in few weeks or months, requiring corticosteroids only occasionally.