Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a self-limited disease of complex and unclear etiology. Clinically and microscopically, it may mimic malignant lymphoma and other non-malignant diseases. Recognition of this entity is crucial, as mistaking this disease as lymphoma has major clinical consequences. Although KFD is a well-recognized entity in the literature, many clinicians and pathologists are still unaware of its existence. In this article, a review of KFD is provided with special emphasis on the pathogenesis and pathological differential diagnosis of this disease.