A 60‐year‐old woman was referred to our clinic for evaluation of her rapidly progressive dyspnea, and she had no previous history of heart disease. A murmur was noted on her examination, and transthoracic echocardiography was so difficult to be performed due to poor acoustic windows so she was referred to do a transesophageal echocardiography that showed an ostium primum atrial septal defect (ASD) with left‐to‐right shunt and a quadrileaflet mitral valve with severe regurgitation. Later on, she underwent surgery with ostium primum ASD closure by a patch and double cleft repair by suture after right heart catheterization.