Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is an extremely rare, self-limiting disorder which typically presents with fever and painful, unilateral cervical lymphadenopathy in previously healthy individuals. Here, we describe a case of KFD which initially presented with fever of unknown origin. Due to its non-specific symptoms and low incidence, KFD poses a diagnostic conundrum for clinicians. Awareness of this disease entity is the key for prompt diagnosis and treatment.