BACKGROUND The study aim was to investigate the immediate and long-term results of balloon aortic valvuloplasty (BAV) in young patients (aged ≤20 years) with congenital bicuspid aortic valve presenting with severe aortic stenosis. METHODS The study involved a single tertiary care center-based retrospective data analysis of immediate and long-term outcomes in patients following balloon valvuloplasty between 2000 and 2011. A total of 92 young patients (mean age 12.7 years; 95% CI 8.3-16.7 years) with aortic stenosis due to congenital bicuspid aortic valve and who underwent BAV were studied. RESULTS The mean follow up period was 5.7 ± 1.3 years. Intervention resulted in successful BAV (≥50% reduction in baseline gradient) in 79 patients (85.9%; group A) and partially successful BAV (<50% reduction in baseline gradient) in eight patients (8.7%; group B). BAV failed in five patients (5.4%; group C). The mean left ventricular systolic pressure was decreased from 155.6 mmHg (95% CI 132.7-186.3 mmHg) to 100.9 mmHg (95% CI 82.1-119.6 mmHg; p <0.001), and the mean aortic valve gradient from 40.7 mmHg (95% CI 25.12-56.22 mmHg) to 17.2 mmHg (95% CI 15.83- 21.23 mmHg; p <0.001). Mean changes in aortic valve area and mean gradient were significantly different between successful BAV groups (A and B) and the failed BAV group (p = 0.001). Different grades of aortic regurgitation were noted in 32 patients (34.78%) after BAV (severe regurgitation in 2.18%). A post hoc analysis showed sustained gradient reductions at one- and five-year follow up investigations (p <0.05). The need for surgery was much lower in the successful BAV groups. CONCLUSIONS The success of BAV in bicuspid aortic valve stenosis in younger patients was approximately 85%. Although 10% of patients developed re-stenosis and required surgical intervention, the majority did well during the follow up period. It was concluded that BAV is a viable option in adolescents and young children with bicuspid aortic valve without severe calcification.