There are several conditions where the function of the aortic valve can be compromised in the pediatric population. The aortic valve is composed of three leaflets which are thin and mobile and are attached to the aortic sinuses. Each leaflet is made up of connective tissue, forming a highly ordered network of extracellular matrix components. Together, this enables the aortic valve to open and close more than 100,000 times throughout the day. However, there are conditions where the structure of the aortic valve can be compromised resulting in its function being affected. Conditions such as congenital valvular aortic stenosis and abnormal valve morphology including bicuspid valves often necessitate intervention to improve symptoms and quality of life in children. Other conditions which result in requiring surgical intervention include infective endocarditis and trauma. In this article, we present the common forms of aortic valve disease in the pediatric population and the clinical presentation and pathophysiology of these. We also discuss the range of management options including medical management and percutaneous intervention. Surgical interventions such as Aortic annular enlargement techniques, the Ross procedure and the Ozaki procedure will also be discussed. The effectiveness, complications and long-term outcomes associated with these methods will be explored.