Kawasaki disease (KD) is a systemic vasculitis that predominantly affects infants and young children. Asian race has been described as an important factor for the development of this disease. We reported our experience in a cohort of patients with KD. Clinical and laboratory data from initial presentation and follow-up visits were reviewed in 88 patients with KD treated at Children's Hospital of New Orleans between March 1993 and November 2004. Forty-nine (56%) patients were African American, 35 (40%) Caucasian, two (3%) Asian and two (3%) Hispanic. Coronary artery aneurysms (CAAs) were detected in 15 (17%) patients. CAAs were less frequent in African-American patients compared to Caucasians [relative risk (RR)=0.2, 95% CI: 0.04400-0.8405, p=0.0164]. Eighty-three patients responded to one dose of intravenous human immunoglobulin (IVIG), five patients responded to a second infusion of IVIG, and two who were IVIG resistant responded only following methylprednisolone pulse. Hemoglobin (Hb) levels of <10 g/dl and presence of polymorphic rash were significantly correlated with development of CAAs. No deaths were observed.