BACKGROUND Kikuchi-Fujimoto disease (KFD) is a rare self-limited necrotizing lymphadenitis which is likely under-diagnosed in pediatric patients who present with fever of unknown origin and lymphadenopathy. Definitive diagnosis is challenging as it requires an invasive open lymph node biopsy or lymph node needle aspiration cytology that shows pathologic findings of histiocytic necrotizing lymphadenitis. CASE REPORT We report the case of one of the youngest patients diagnosed with KFD in the United States, at the age of 7 years. KFD has a higher prevalence in patients of Asian descent, but this patient was an African American. This case report shows the often convoluted and complicated course these patients undergo with their presenting complaints of fever of unknown origin and lymphadenopathy and highlights particular clinical findings that suggest KFD. CONCLUSIONS This patient is one of the youngest persons diagnosed with KFD in the United States, with an atypical ethnic background. It is likely that KFD is under-recognized and under-diagnosed in this population. With a broad differential diagnosis for fever of unknown origin and lymphadenopathy, awareness of KFD as a potential diagnosis may reduce other unnecessary investigations. The increased risk of patients with KFD of developing systemic lupus erythematosus (SLE) accentuates the importance of an accurate diagnosis and appropriate referral for heightened surveillance after recovery.