Lutembacher syndrome is characterized by the association of mitral stenosis and atrial septal defect (ASD), usually of the ostium secundum type. The association between superior vena cava-type ASD and partial anomalous pulmonary venous connection is unusual and there are few descriptions in the literature. We report this condition in a 24-year-old woman who was admitted to the hospital with a 1-year history of progressive dyspnea and describe the successful surgical repair following mitral commissurotomy and tunneling of the anomalous pulmonary veins to the left atrium, which determines the closure of the ASD and tricuspid repair.