Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disease. It mostly affects young adults in their 20-30 years of age and has female predominance. KFD also affects children but the overall population of pediatric patients has not yet been defined in the literature. Its etiology is unclear and a role of the autoimmune system in the pathogenesis is hypothesized. KFD is well known for its association with systemic lupus erythematosus (SLE). Cervical lymphadenopathy is the most prominent and encountered sign and should be histopathologically differentiated from lymphoproliferative, autoimmune, and infectious diseases. We report a 14 years old girl with persistent cervical lymphadenopathy and fever, diagnosed initially with bacterial lymphadenitis and later her diagnosis was corrected as KFD after histopathology report. We herein provide a brief review about KFD and emphasize on the importance of prompt histopathological examination for patients presented with clinically significant lymphadenopathy and unfavorable clinical features in order to make timely accurate diagnosis.