We evaluated the significance of corticosteroid therapy on 47 new patients with Vogt-Koyanagi-Harada disease examined in the Uveitis Survey Clinic of the Hokkaido University Hospital. All patients were treated with topical corticosteroids, with or without systemic corticosteroids. 18 patients received systemic corticosteroid as pulse therapy, 20 patients received high-dose corticosteroid starting with prednisolone 200 mg, 2 patients received conventional-dose corticosteroid and 7 patients received no systemic corticosteroid therapy. When we evaluated the results 6 months after the initiation of treatment, anterior chamber inflammation was significantly less in patients with pulse and high-dose corticosteroid therapy than in those without systemic corticosteroid therapy. Furthermore, final visual acuity was significantly better in patients with pulse and high-dose corticosteroid than in those without them. However, there was no significant difference between patients with pulse therapy and those with high-dose corticosteroid therapy. The findings are in support of systemic corticosteroid therapy (pulse and high-dose) in the treatment of Vogt-Koyanagi-Harada disease.