Background: Kawasaki Disease (KD) is an acute febrile vasculitis of early childhood and the leading cause of acquired heart disease in children. The most important complication of KD is coronary artery aneurysm that may lead to thrombosis and myocardial infarction, occurring in 25% of untreated cases. Fortunately, timely treatment decreases it to 2 -4%. There is no specific test for the diagnosis of KD, which is entirely based on clinical and laboratory criteria. Objectives: Since KD is very common in Iran and other Asian countries, it is necessary to get a clear picture on the frequency of KD related anomalies in the clinical and laboratory findings as well as the frequency of coronary aneurysms in our region. Patients and Methods: We retrospectively studied the medical records of all children discharged from the Besat Hospital, Hamedan, Iran, with a final diagnosis of KD, from 2004 to 2013. Outpatient documents were also studied. Demographic data, clinical features, laboratory and echocardiographic findings were also recorded onto the forms. Results: Seventy four patients, 43 (58.1%) males and 31 (41.9%) females, were discharged with a final diagnosis of KD, 77% of which were less than 5 years old. No seasonal variability was noticed in the study population. Among all cases, 44 (59.5%) were diagnosed as complete and 30 (40.5%) as incomplete KD. Conjunctivitis, considered as the most common clinical finding, occurred in 79% of patients, whereas peripheral erythema was the least common, corresponding to 23% of cases. Finger desquamation and leukocytosis occurred in 54% and 49.3% of cases, respectively. Amongst the timely-treated patients, 11 (14,8%) developed coronary artery aneurysm. Conclusions: Sex, age, and seasonal patterns of KD in Hamedan Province, IR Iran, are similar to other regions. However, finger desquamation is less prevalent, diminishing its value as a measure for patients' follow up. The most common supplementary laboratory criteria were leukocytosis, thrombocytosis and anemia, which were determined by a complete blood count. Coronary artery aneurysm in timely-treated patients was far more common compared to other studies, which may indicate the need for revising the golden time for initiating the treatment.