Atrial septal aneurysm (ASA) is a known morphologic abnormality and has been largely investigated with two-dimensional echocardiography. Its association with other congenital or acquired diseases has also been described. However, the presence of ASA and concomitant Ebstein's anomaly in the same patient is a very unexpected finding in an adult subject. We therefore present the case of a young asymptomatic woman with this association.