Liver involvement is usually a minor manifestation of Kawasaki disease and includes hepatobiliary dysfunction or gallbladder hydrops. We describe here an unusual case of jaundice revealing an adult onset Kawasaki disease. An 18-year-old man presented with abdominal pain and jaundice associated with cholestasis as the initial manifestation of Kawasaki disease. Abdominal evaluation (ultrasonography and CT-scan) did not find abnormality. Other signs typical of the Kawasaki disease occurred a few days later and permitted diagnosis. With aspirin and intravenous immunoglobulins, outcome was favorable without any cardiovascular complication. Our case suggests that Kawasaki disease should be added to the etiological list of painful febrile icterus in young patients.