Kikuchi-Fujimoto disease (KFD) is subacute necrotizing lymphadenitis characterized by fever, leukopenia and cervical lymphadenopathy. There are few reports on the clinical characteristics and laboratory findings of KFD in the pediatric literature. In this study, we evaluate the characteristics and outcome of KFD in children. A total of 412 patients were studied with fever and peripheral lymphadenopathy at Pusan National University Hospital from January 1998 to December 2003. Among the total 412 there were 16 patients diagnosed with KFD by lymph node biopsy. We analyze the clinical, laboratory and outcome after review of the medical records retrospectively. The mean age of the patients was 10.6 +/- 3.4 yr (range: 4-17 yr). The male to female ratio was 1:1. Almost all patients, except two, had cervical lymph node swelling. The size of the involved lymph node was less than 4 cm in the greatest dimension in 75% of the patients. All the children had fever as the chief complaint and the mean duration of the fever was 17.7 +/- 11.2 days (range: 2-122 days). Approximately 87% of the patients had leukopenia (WBC < 4000/mm(3)) and 43.8% of the patients had a mild increase in the transaminases on liver function testing. A total of 8 out of the 16 patients were initially misdiagnosed as an infectious disease and treated with antibiotics which caused prolonged hospitalization for most patients. Six patients were treated with prednisone and the prolonged fever subsided immediately after steroid therapy. KFD should be considered in the differential diagnosis of prolonged fever in children with cervical lymphadenopathy. Early cervical lymph node biopsy is necessary to minimize inappropriate examinations and treatments in such cases.