Unicuspid aortic valve is a rare congenital abnormality with a prevalence of 0.02% in the adult population. Left ventricular noncompac tion is another congenital anomaly with an incidence of 0.05%. Unicuspid aortic valve with severe aortic regurgitation and left ventricular noncompaction was found in a 15-year-old deaf male patient who presented with shortness of breath, orthopnoea and intermittent palpitations