The vast majority of atrial myxomas are readily apparent on standard transthoracic echocardiography. We present the case of a young woman with known history of familial myxoma, in whom a large left atrial mass was identified on transthoracic echocardiography. Transesophageal study more clearly delineated the extent and origin of the tumor. In addition, an unsuspected tumor in the right ventricle was also identified. These findings were confirmed at surgery, and both masses were excised. The advantages of transesophageal echocardiography in evaluation and management of this unusual subset of patients with cardiac tumor are discussed.