Congenital anomalies of the aortic valve frequently necessitate intervention in childhood. The most common aortic valve pathologies present in childhood are aortic stenosis and insufficiency. Presentation of aortic valve disease depends on severity and presence of concomitant syndromes and valvular disorders. Treatment options are largely categorised as medical, percutaneous repair or surgical repair and replacement. Surgical techniques have been refined over the last few years making this the mainstay of treatment in paediatric cases. Whilst repair is considered in most instances before replacement, there are substantial limitations which are reflected in the frequency of reintervention and restenosis rate. Replacements are typically undertaken with tissue or mechanical prosthesis. The current gold-standard aortic valve replacement surgery is called the Ross procedure—where replacement is undertaken with a competent pulmonic valve and a simultaneous pulmonary homograft.Conclusion: In this review, we aim to outline the various surgical options and discuss efficacy and complications of various interventions. What is Known: • Congenital aortic valve defects repair options medically and surgically What is New: • Comparisons between surgical options for aortic valve repair including efficacy, risks and long-term outcomes.