Coarctation of the aorta (CoA ) is a well-known congenital heart disease (CHD) , which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV) and ventricular septal defect. BAV is the most common CHD whereas Pacman heart (incomplete muscular ventricular septal defect) is rare congenital deformity. We found a coexistence of Pacman heart, CoA and BAV in a 21- year- old male patient presenting with headache and high blood preesure. This extremely rare combination of these three congenital cardiac abnormalities occurring in the same patient has not been reported yet.