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Kawasaki disease and hepatobiliary involvement: report of two cases. BACKGROUND: Kawasaki disease (KD) without affection of the coronary artery system is rare. Optic nerve pathology together with KD has not been described earlier. CASE PRESENTATION: We present one case of KD in a 12-year-old girl predominantly with prolonged cholestasis, and a second case of multiple recurrent KD in a 9-year-old boy with hepatomegaly and ischemic optic nerve neuropathy. The coronary artery system was not involved in either case. CONCLUSIONS: KD warrants rapid diagnosis and immediate specific treatment in order to prevent the high risk of coronary artery aneurysm and stenosis.