A 15-year-old boy was evaluated for the new onset of dyspnea. He had no significant medical or surgical history. On echocardiography, he was found to have a direct communication between the left ventricle and the right atrium through the atrioventricular (AV) septum (a rare type of Gerbode defect) with an associated cleft in the anterior mitral leaflet. The combination of direct type Gerbode defect with the cleft of the anterior mitral leaflet is extremely rare.
