Kikuchi-Fujimoto disease (KFD) is a rare cause of cervical lymphadenitis which mostly affects Asian women but is sometimes observed in the pediatric population. This study analyzed the clinical manifestations and disease course in children with KFD. Retrospective chart review and telephone interview were used to collect data for 13 children (8 boys and 5 girls) with a diagnosis of KFD from January 1988 to January 2003. Involvement of the posterior cervical lymph nodes was found in 12 patients, leukopenia in 9, and all patients had C-reactive protein less than 5 mg/dL. A high antinuclear antibody titer was associated with a more protracted and complicated course. Five of the 13 patients had new symptoms compatible with an autoimmune process during follow-up, with neurological symptoms the most common. In conclusion, the risk of evolution into an autoimmune syndrome in pediatric KFD patients is high, and careful long-term observation is mandatory.