BACKGROUND Balloon aortic valvuloplasty (BAV) is a well-established treatment modality for congenital aortic valve stenosis. AIM To evaluate the role of rapid right ventricular pacing (RRVP) in balloon stabilization during BAV on aortic regurgitation (AR) in pediatric patients. METHODS A systematic review of the MEDLINE, Cochrane Library, and Scopus databases was conducted according to the PRISMA guidelines (end-of-search date: July 8, 2020). The National Heart, Lung, and Blood Institute and Newcastle-Ottawa scales was utilized for quality assessment. RESULTS Five studies reporting on 72 patients were included. The studies investigated the use of RRVP-assisted BAV in infants (> 1 mo) and older children, but not in neonates. Ten (13.9%) patients had a history of some type of aortic valve surgical or catheterization procedure. Before BAV, 58 (84.0%), 7 (10.1%), 4 (5.9%) patients had AR grade 0 (none), 1 (trivial), 2 (mild), respectively. After BAV, 34 (49.3%), 6 (8.7%), 26 (37.7%), 3 (4.3%), patients had AR grade 0, 1, 2, and 3 (moderate), respectively. No patient developed severe AR after RRVP. One (1.4%) developed ventricular fibrillation and was defibrillated successfully. No additional arrhythmias or complications occurred during RRVP. CONCLUSION RRVP can be safely used to achieve balloon stability during pediatric BAV, which could potentially decrease AR rates.