Congenital aortic valve stenosis in children, one of left ventricular outflow tract obstructive lesions, is difficult to operate with high risk of re-intervention. It becomes one of the difficulties in the field of children's heart disease. According to the peak gradient pressure across aortic valve, it can be divided into mild stenosis (<50 mm Hg), moderate stenosis (50-75 mm Hg) and severe stenosis (>75 mm Hg). Emergency or limited surgery is required for the patients with heart failure and patent ductus arteriosus (PDA)-dependent circulation, and patients with peak pressure gradient of 50 mm Hg or higher, or with obvious clinical symptoms should undergo surgery. The main surgical procedures include balloon aortic valve dilatation, suigical aortic valvulotomy, aortic valve reconstruction with pericardium, other aortic valvuloplasty, Ross operation and valve replacement, and aortic annulus enlargement if necessary. Heart transplantation should be considered in case of unrepairable severe lesions and poor results of valve repair. Fetal intervention remains controversial with high incidence of related complications. Aortic valve stenosis in children is a lifelong problem. To release valve stenosis appropriately and to promote children’s growth and development are the main purposes of surgery, but some children eventually need more surgeries and finally valve replacement. The long-term complications, survival rate and reintervention rate still need more attention.