The purpose of this study was to describe the prevalence, clinical characteristics, and causes of vision loss in children with Vogt-Koyanagi-Harada disease seen at a uveitis referral center in South India. Charts of patients with Vogt-Koyanagi-Harada disease examined in the uveitis referral clinic of Aravind Eye Hospital between January 1998 and December 2007 were reviewed. A subset of patients <or=16 years of age was identified, and the clinical characteristics and causes of vision loss were evaluated. Vogt-Koyanagi-Harada disease was diagnosed in 267 of 22,959 patients (1.2%) during the study period. Twenty-two children (8.2%) were identified, including 13 girls (59.1%) and 9 boys (40.9%). Age at presentation ranged from 8 years to 16 years, with a mean and a median of 12.6 and 13.5 years, respectively. The most common complaints were blurred vision (39 eyes, 88.6%) and eye redness (36 eyes, 81.8%). Fifteen (68.5%) children had headaches, 6 (27.3%) developed meningismus and alopecia, 4 (18.2%) developed poliosis and vitiligo, and 3 (13.6%) had tinnitus or dysacusis. Initially, all were treated with oral prednisone, but 12 (54.6%) required methotrexate, 5 (22.7%) required azathioprine, and 2 (9.1%) required cyclophosphamide. Seventy-five percent of eyes had a final visual acuity of >or=20/40, whereas 13.6% had a final visual acuity of <or=20/200. Vogt-Koyanagi-Harada disease is an uncommon cause of uveitis in children. The clinical characteristics of pediatric Vogt-Koyanagi-Harada disease in South India resembled those described in cohorts from other regions. Although children in our cohort tended to do well with prompt diagnosis and treatment, long-term vision loss can occur.