Acute cholestasis: atypical onset of Kawasaki disease

Atypical onset of Kawasaki disease (KD) is a frequent problem leading to diagnostic mistake. Acute cholestasis and liver involvement occur occasionally as minor manifestation of KD. We report the case of a 6-year-old boy presenting fever, jaundice, abdominal pain, and ascites who subsequently developed typical KD clinical pattern just at the same time of echocardiographic coronary arteries anomalies. Abdominal radiological evaluation was normal and seroimmunologic markers resulted negative. Shortly after intravenous immunoglobulin and acetylsalicylic acid administration the clinical features disappeared. KD should be considered in differential diagnosis in children with cholestasis, abdominal pain and fever of unknown etiology.