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Kawasaki disease predominantly affects children younger than 5 years. Coronary artery aneurysms were found in around 20% of untreated patients. We report on a case of a 10-year-old boy who had atypical presentation of Kawasaki disease with significant hepatobiliary dysfunction, including hepatomegaly and jaundice, and persistent fever. He did not have conjunctivitis until the 8th day of fever, and periungual desquamation and strawberry tongue until the 13th day of fever when Kawasaki disease was diagnosed. Echocardiography revealed multiple coronary artery aneurysms. Such atypical clinical pictures of Kawasaki disease may cause delay in the treatment, and the fatality rate can be increased. So, a persistent fever with jaundice should evoke the differential diagnosis of Kawasaki disease.

Kawasaki disease presenting with hepatitis and prolonged fever: report of one case.