Background: Kawasaki Disease (KD) and acute rheumatic fever are the two leading causes of acquired heart disease in children in the developing countries. Objectives: To determine the epidemiology of KD and its short-term outcome in Malaysian children. Materials & Methods: A retrospective study of patients with a diagnosis of KD at the Kuala Lumpur Hospital from January 1999 to December 2003. Results: 84 patients with KD were seen over the 5 year period. Of these, 52 (61.90%) were male and 32 (38.10%) female. Malays comprised 51 (60.71%), Chinese 30 (35.71%) and Indian 3 (3.57%). Their ages ranged from 2 months to 11 years 1 month old. There were 25 (29.76%) patients less than one year old, 50 (59.52%) aged 1-4 years, 4 (4.76%) aged 5-7 years and 5 (5.95%) were more than 7 years old. Echocardiographic examination during the acute phase showed that 24 patients (28.57%) had coronary artery dilatation; 23 had mild dilatation and one had giant aneurysm involving both coronary arteries. Echocardiographic examination at 8 weeks showed that 5 (21.74%) of the 23 patients with mildly dilated coronary arteries had resolved; the bilateral giant coronary aneurysms remained the same. There was no death due to KD over the 5 year period. Conclusion: KD occurred most commonly in children aged 1-4 years old with a peak at 17 months. There was male preponderance with a male to female ratio of 1.6:1. Despite immunoglobulin therapy, 29% of patients had coronary artery involvementduring the acute phase; 22% of those with mild coronary artery dilatation resolved at 8 weeks after disease onset.