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Aortic valve (AV) prolapse and subsequent aortic regurgitation (AR) are two complications of ventricular septal defects (VSD) that are located close to or in direct contact with the AV. This finding is one of the indications for surgical VSD closure even in absence of symptoms in order to protect the AV integrity. Goal of our study was to assess the outcome, and to identify the predictors for improvement or progression of AR after surgical repair. A retrospective study of all children with VSD and AV prolapse who underwent cardiac surgery at King Abdulaziz Cardiac Center in Riyadh between July 1999 till August 2013. A total of 41 consecutive patients, operated for VSD with prolapsed AV, with or without AR, were reviewed. The incidence of AV prolapse in the study population was 6.8% out of 655 patients with VSD. Thirty-six (88%) patients had a perimembranous VSD and 4 had doubly committed VSD. Only one patient had an outlet muscular VSD. Right coronary cusp prolapse was found in 38 (92.7%) patients. Preoperative AR was absent in 5 patients, mild or less in 25 patients, moderate in 7 and severe in 4 patients. Twenty six patients showed improvement in the degree of AR after surgery (Group A), 14 patients showed no change in the degree of AR (Group B) while only one patient showed progression of his AR after surgery. Those with absent AR before surgery remained with no AR after surgery. Improvement was found more in those with mild degree of AR preoperatively compared to those with moderate and severe AR. Female gender also showed tendency to improve more as compared to male. Early surgical closure is advisable for patients with VSD and associated AV prolapse in order to achieve a better outcome after repair and to prevent progression of AR in future.