Kawasaki disease is an acute febrile illness of childhood that affects usually children younger than 5 years of age. The diagnosis is based on typical clinical features. Atypical or incomplete forms of Kawasaki disease can be observed in almost 20% of patients and at onset is a clinical challenge leading to diagnostic and therapeutic delays with higher risk of coronary abnormalities. We report the case of a 13-year-old boy who presented with febrile cholestasis, abdominal pain and early coronary anomalies that was diagnosed with Kawasaki disease. Atypical findings of our case at onset were the age, the clinical presentation and the timing of appearance of coronary anomalies. A high index of suspicion of Kawasaki disease should be maintained in patients presenting with febrile cholestatic jaundice.