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Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis) is a benign self-limiting disease with unknown aetiology. It is characterized by enlargement of lymph nodes, which may be tender and can be accompanied by fever and other upper respiratory tract symptoms. Diagnosis is usually rendered with excisional biopsy of lymph nodes and through histopathological findings. There is not any treatment for this disease. This unknown disease due to difficulties in diagnosis was discussed with clinical and pathological findings.