This study was conducted to determine the epidemiology, clinical manifestations, cardiac involvements and laboratory findings of Kawasaki disease (KD) in Fars Province, Iran from January 1991 to December 2002. One hundred and thirteen patients with KD were confirmed. This syndrome occurred mostly in males (ratio=2.1:1). The most commonly affected age group was one to five years old. There was no secondary case in families, none of the patients were related and recurrence of disease was seen in only one patient. Prolonged fever and thrombocytosis were significant risk factors for developing coronary artery disease (p<0.05, respectively). Among the 10 patients with cardiac involvement, seven patients had dilated coronary arteries (>4 mm) and three patients had aneurysm of coronary artery in their first echocardiography. It has been suggested that a high index of clinical suspicion of KD could improve diagnosis and implementation of preventive treatment.