Kawasaki disease (KD) is the self-limited and multisystem vasculitis which accompanies many complications. Ophthalmic findings in KD are bilateral conjunctival injection, iridocyclitis, superficial keratitis, vitreous opacities and subconjunctival hemorrhage. Optic disc swelling is a rare ophthalmic complication in KD. We describe a 3-year-old boy who presented with 7 days of fever, both conjunctival injection without discharge, and right cervical lymph node enlargement of more than 1.5 cm. He was diagnosed as incomplete KD. He had no ocular symptom except bilateral conjunctival injection. On ophthalmic examination, he was diagnosed by anterior uveitis with optic disc swelling. The brain magnetic resonance imaging was performed and revealed no evidence of increased intracranial pressure. Echocardiography revealed the dilated right coronary artery up to 3.4 mm. Fever subsided and optic disc swelling was completely improved after intravenous immunoglobulin (2 g/kg) treatment. Optic disc swelling is a rare ophthalmic complication in KD.