: Ocular involvement in the acute phase of Kawasaki disease typically involves the anterior segment of the eye, and its associated treatment outcomes are generally excellent. However, there are rare reports of posterior segment involvement, and these are associated with poorer outcomes. This study reviewed six patients with recently reported blindness and/or ocular posterior segment involvement in the acute phase of Kawasaki disease. The median age of these patients was 8.5 years old (range: 6-12 years old). The ocular symptoms were photophobia, reduction of vision, blindness, and ocular pain. Five patients exhibited optic disc involvement and three patients showed retinal vessel involvement. All patients received intravenous immunoglobulin therapy and two patients received systemic corticosteroid administration. The visual acuity outcomes of treatment were excellent in five of the patients. However, in one patient there was still no light perception in the affected eye at two months after the initial onset of the disease. This was associated with the possible obstruction of the ophthalmic artery due to vasculitis. Older children may have a higher associated risk of blindness and ocular posterior segment involvement in the acute phase of Kawasaki disease.