We present a case of prenatal diagnosis of critical congenital aortic valve stenosis with progressive systolic left ventricular failure. An ultrasound-guided balloon aortic valvuloplasty was performed at 28 weeks of gestational age because of left ventricular dysfunction associated with signs of fetal heart failure. There were no significant post-procedural complications and the pregnancy was carried to term with elective cesarean section at 38 weeks of gestational age. At birth, an echocardiogram showed severe aortic valve stenosis with global hypokinesia of the left ventricle. Therefore a percutaneous balloon aortic valvuloplasty was repeated through transseptal approach with prompt improvement of the antegrade aortic flow and of the left ventricular systolic function. The baby is currently 2 months old and he is doing fine.