Background: Kawasaki disease (KD) is described as a life-threatening vasculitis, which mostly develops in children below five years of age and is diagnosed according to clinical criteria. It is also a common rheumatologic disorder in Iran. Objectives: The present study aimed at determining the clinical and demographic characteristics of KD patients in Iran. Methods: We retrospectively assessed69cases of KDinanIranian pediatric populationfromMarch2014 toMarch2018. The Japanese Kawasaki Disease Research Committee guidelines were used as the diagnostic criteria for typical KD. Incomplete or atypical KD was diagnosed in patients with coronary artery changes, but without all the criteria for KD. Results: In this study, 69 patients were recruited, with a male-female ratio of 1: 8. Overall, 64% (n, 44) and 36% of children met the criteria for typical and atypical KD, respectively. Also, echocardiographic abnormalities were reported in eight patients (12%). Coronary artery aneurysm was found in 2% of patients, while other cardiac abnormalities were found in 12% of patients. The male-female ratio of coronary artery anomalies and other cardiac abnormalities was 3: 1. Polymorphic exanthema was the most common clinical manifestation. The erythrocyte sedimentation rate 40mm/hwas the mostcommonlaboratory finding, while skin desquamation was the most common complication in other organs. Conclusions: KD is not uncommon in MARKAZI PROVINCE, Iran. In this study, distribution of demographic characteristics was not similar to reports from other countries. Also, clinical findings, age and gender distribution, laboratory findings, and complications in other organs were not similar to previous reports. The incidence of typical and atypical KD was different in this region, especially in terms of complications, such as cardiac and gastrointestinal complications.