Lutembacher syndrome is an unusual clinical entity of congenital secundum atrial septal defect in combination with rheumatic mitral stenosis. Although this classic form is seldom seen by the adult cardiologist, spontaneous Lutembacher syndrome as discussed later or the iatrogenic variant is not infrequently encountered. The pathophysiologic, clinical, and hemodynamic differences of mitral valve disease in the presence of atrial septal defect compared with isolated mitral stenosis are highlighted in this case review. Special emphasis has also been given to echocardiographic evaluation of this syndrome complex, particularly in the setting of percutaneous mitral valvuloplasty, which produces the iatrogenic form of Lutembacher syndrome.