Unicuspid aortic valve (UAV) is a very rare congenital heart defect with an ill-defined natural history. It’s very important to diagnose early as UAV has a risk of sudden cardiac death and association of other congenital anomalies. Here we present a case of unicommissural UAV with severe AS, severe LV dysfunction diagnosed by colour doppler TT echo, successfully treated with balloon valvoplasty (BAV).