Rheumatic heart disease (RHD) causes significant morbidity among children of low and middle-income countries. We aimed to assess characteristics and outcomes of Indian children enrolled in a tertiary care hospital RHD registry.Pediatric patients (≤18years) were prospectively enrolled over three years. We evaluated their clinical and echocardiographic characteristics, valvular interventions, adherence to penicillin prophylaxis, and mortality.451 patients were enrolled in the registry (mean age 12.2±3.2years, 34% females). Females had greater burden of exercise intolerance than males (26.8% NYHA classes III–IV versus [vs] 13.8%, p<0.01). Among 388 patients with no prior interventions, mitral regurgitation was the most common lesion (48.7%). 279 patients (71.9%) had severe valvular disease and this proportion was higher in females than males (80.9% vs. 67.6%, p<0.01). 248 patients received AHA/ACC class I recommendation for valvular interventions and 53 (21.4%) underwent an intervention during 11month (median) follow-up. Age (adjusted odds ratio [aOR] 1.18, 95% confidence interval [CI] 1.03–1.36), NYHA classes III–IV (aOR 4.96, 95% CI 2.51–9.60), mitral stenosis (aOR 37.01, 95% CI 6.96–196.74), and mixed mitral valve disease (aOR 2.79, 95% CI 1.03–7.56) were predictive of undergoing an intervention. Mean adherence to penicillin prophylaxis among 274 patients with clinic follow-up was 93.6%. 14 patients (3.1%) died during follow-up.Females had greater disease severity in an Indian pediatric RHD registry. Adherence to penicillin prophylaxis in the cohort was good. After 11months follow-up, one in five patients underwent a guideline-recommended intervention. Mortality was high during this short follow-up period.