Forty-one children with a clinical diagnosis of Kawasaki syndrome had a pediatric ophthal-mologic examination performed at a mean time of 8.6 days after onset of fever. Twenty-seven of 41 patients (66%) had evidence of anterior uveitis by slit lamp examination. Bilateral involvement was noted in 25 of 27 patients (97%). Punctate keratitis was evident in 5 of 41 patients (12%). Three of these five had coexisting uveitis. The incidence of anterior uveitis in patients examined during the first week of their illness was 83% (20 of 24 patients), compared to an incidence of 41% (7 of 17) in patients first examined greater than 1 week after onset of disease (P = 0.004). There was no difference in the uveitis, vs. the non-uveitis groups with respect to age, sex, race or subsequent aneurysm formation as a complication of their Kawasaki syndrome. The mean erythrocyte sedimentation rate was not different in the two groups. Anterior uveitis is a common finding early in the course of Kawasaki syndrome. Slit lamp examination may be a helpful clinical tool in identifying patients with Kawasaki syndrome, and uyeitis should be considered for inclusion in the Centers for Disease Control case definition of Kawasaki syndrome.