Left ventricular-right atrial communications, known as Gerbode-type defects, are rare intracardiac defects that can be either congenital or acquired. Acquired forms arises from endocarditis, trauma, mitral or aortic valve replacement and myocardial infarction. In this case, a forty-seven year-old man with resting dyspnea and fever was diagnosed to have infected congenital bicuspid aortic valve resulting in severe acute aortic regurgitation and he also was diagnosed to have a Gerbode-type defect which were demonstrated by the color doppler method and realtime three-dimensional echocardiography. The causasive organism was Streptococcus mitis. After sufficient antibiotics therapy, aortic valve replacement was performed and the atrioventricular portion of the membranous septum was closed directly with mattress suture. We also review the anatomical features and hemodynamic consequences of such a defect.