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.