Kawasaki disease (KD) has not been frequently reported from developing countries, especially from India. In this series from a tertiary level center in North India, we report on the clinical features and management of patients with KD seen between January 1994 to November 2004. KD was diagnosed on the basis of standard diagnostic criteria. Investigations included work-up for other causes of fever along with chest X-rays, electrocardiograms, and 2-D echocardiography. Thallium scintigraphy and coronary angiography were performed in 20 and 3 cases, respectively. Sixty-nine children (49 boys and 20 girls) fulfilled the diagnostic criteria. Mean age at diagnosis was 4.9 +/- 3.0 years (range 0.3-14 years) and as many as 23 cases (33.3%) were above 5 years of age. Clustering was seen during the winter months. Extreme irritability, out of proportion to the degree of fever, was a characteristic feature. Redness of the lips and tongue was common but rash was seen only in 43 cases and lymphadenopathy in 47 cases. Thrombocytosis was present in 52.2% of the patients. Sixty-four patients received intravenous immunoglobulin. Cardiac abnormalities included extrasystoles in 1, coronary artery dilatation in 5, valvular regurgitation in 3, and perfusion defects on thallium scintigraphy in 4. There was no mortality. KD appears to occur at an older age in Indian children as compared to reports from Japan. Irritability is a characteristic clinical finding. Cardiac abnormalities are frequent during the acute stage but regress gradually. The disease needs to be considered in the differential diagnosis of all children with persistent unexplained fever.