The cardiovascular complications directly related to the cause of death are associated with more than 90% of the Marfan syndrome. Aortic aneurysm with rupture could occur because of dilatation of aorta due to defect of media. Aortic and mitral insufficiency, mitral valve prolapse, bacterial endocarditis, arrhythmia, and aneurysm of interatrial septum are also frequently observed.

A few literatures were reported domestically pertaining to the Marfan syndrome thus far, and there are increased discoveries of cardiovascular complications of the syndrome with the application of echocardiogram.

However, the Marfan syndrome with LA myxoma has never been reported both domestically and internationally(INDEX MEDICUS, 1966-1989). Therefore we hereby report a case of the Marfan syndrome with LA myxoma from the observation of a patient who was admitted to Eulji General Hospital at Taejeon i August of 1989.