OBJECTIVE To identify the risk factors associated with the development of coronary artery lesions (CAL) in pediatric patients with Kawasaki Disease (KD). METHODS A retrospective chart review of the medical records of pediatric patients diagnosed with KD who were admitted to King Abdulaziz University Hospital, Jeddah, Saudi Arabia between January 2001 and December 2011 was performed. Odd ratio (OR) with 95 % Confidence interval (CI) was measured for each risk factor for CAL. Data were presented as frequencies (percentages). A P-value less than 0.05 was considered statistically significant. RESULTS A total of 44 patients were included in the study. Males were predominant, 27 (61.4%). The mean age of the patients was 26.7 months (range 1.5-108 months). Typical KD was recorded in 23 patients (52.3%), while coronary artery lesions were found in 26 (59.1%) patients. Coronary artery lesion was more common among patients with atypical KD, males, patients presented with history of fever more than 5 days, and intravenous immunoglobulins (IVIG) infusion after 10 days of start of fever (OR 4.2, 95% CI 1.1-15.3; p =0.01, OR 5.2, 95% CI 1.4-19.5; p =0.01, OR 5.4, 95% CI 1.5-20.1; p =0.01, OR 5.6, 95% CI 1.4-21.9; p =0.6, respectively). CONCLUSIONS Atypical Kawasaki disease should be considered in children and early administration of IVIG reduces the frequency of coronary artery lesions.