Valvular aortic stenosis in pediatric age group is mostly congenital in origin. The aortic valve may be unicuspid, bicuspid, tricuspid or rarely quadricuspid. Left ventricle undergoes concentric hypertrophy secondary to obstruction to its outflow tract. In neonatal aortic stenosis, left ventricle may be hypoplastic. The pathophysiology and clinical presentation vary with the age of onset and severity of obstruction. Echocardiography and Doppler are indispensible for the diagnosis of aortic stenosis and its severity. Cardiac catheterization is not necessary for the diagnosis, however it is performed as a part of balloon aortic valvuloplasty in severe cases. Balloon valvuloplasty is an effective alternative to surgery in pediatric age group. Some of these patients require surgical valve repair or replacement on follow-up. Neonates and young infants with critical aortic stenosis present with cardiogenic shock and need aggressive treatment with prostaglandin E1 infusion along with inotropic support. In experienced hands, balloon valvuloplasty is a safe procedure in neonates and infants with critical stenosis. Patients with mild and moderate aortic stenosis may be left on medical follow-up.