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Kawasaki disease (KD) or mucocutaneous lymph node syndrome is a vasculitis that mostly occurs in young children. Adult-onset KD (AKD) is rare and often misdiagnosed. Here we report a rare case of KD with cholestasis as principal symptom. A 43-year-old caucasianman was admitted to our hospital for high fever, lack of appetite related to nausea and vomiting, headache and significant malaise. Physical examination highlighted fever, increasing jaundice, bilateral laterocervical lymph nodes, erythema of the palms, and strikingly red lips an