We encountered a 78-year-old female with jaundice induced by Lutembacher's syndrome, which, to our knowledge, is the first such case to be reported. Laboratory data and ultrasonographic examination revealed liver congestion with dilatation of the inferior vena cava and hepatic veins. A large atrial septal defect was found using two-dimensional echocardiography; the mitral valve area measured 1.6 cm2 by planimeter. Clinicians should be aware of congenital heart disease as an underlying cause of jaundice.