Purpose : Coxiella burnetii is a well-know causative agent of granuolmatous inflame-mation and an inducer of morphological change and transformation of human B lymphocyte in vitro. Coxiella burnetii manifests with several clinical symptoms depending upon the organs that are involved. We therefore undertook to clarify the association of Kawasaki disease and Coxiella burnetii. Subjects and Methods : The patient’s sera were tested for antibodies specific for Coxiella burnetii, using indirect fluorescent antibody technique(IFA). We compared Coxiella burnetii infection with 3 groups of patients, group 1 (Kawasaki disease), group 2 (other febrile disease) and group 3 (control group). Results : 1) In children with Kawasaki disease (group 1), 93% of the patient tested positive for Coxiella burnetii. 2) In group 2 children, 20% of the patient tested positive for Coxiella burnetii. 3) In group 3 children, 10% of the patient tested positive for Coxiella burnetii 4) There were significant higher positive rate for Coxiella burnetii in Kawasaki disease than group 2 and group 3 (p<0.05). Conclusions : We concluded that our cases of Kawasaki disease were associated with Coxiella burnetii infection. Further studies will be needed to understand the precise role of Coxiella infection in Kawasaki disease.