Infants with Kawasaki disease are at high risk of developing life-threatening coronary complications, yet may elude timely diagnosis because they often lack the full complement of classic clinical features. We retrospectively studied 26,540 children 1 year of age or less who were evaluated at a tertiary care pediatric emergency department in whom a platelet count was performed. Among those infants with fever without a source identified, 8.5% with platelet counts of 800,000 cells/mm(3) or greater had Kawasaki disease compared to 0.4% with platelet counts of less than 800,000 cells/mm(3) (likelihood ratio for Kawasaki disease was 17 [95% confidence interval, 8-34]). Because many infants present atypically, Kawasaki disease should be considered in all children of 1 year or less with prolonged fever, extreme elevation of the platelet count, and no compelling alternative diagnosis.