We present a case of a 36-year-old woman with progressive exertional dyspnoea the New York Heart Association (NYHA) class III, with severe aortic regurgitation (vena contracta 7 mm, prominent holodiastolic reversal in descending aorta), and only mild stenosis (mean pressure gradient 25 mm Hg, aortic valve area 1.62 cm2 ) due to the unicuspid aortic valve combined with coronary-pulmonary fistula.