Sixteen patients aged 22–64 years have undergone removal of atrial myxoma at the Johns Hopkins Hospital. The first two patients had myxomas removed by closed surgical approach, and both died. Fourteen consecutive patients have undergone operation with the aid of cardiopulmonary bypass, and all survived. There has been one late death unrelated to myxoma. Current surgical approach is excision of the tumor and its stalk or base and resection of the portion of intra-atrial septum to ensure complete removal. Recently a new diagnostic technique of two-dimensional phased array real time echocardiography has been used in four patients, including a pregnant woman, to confirm the clinical diagnosis of atrial myxoma. This technique provides a two-dimensional view of both atria and ventricles in cross section or sagittal view. This noninvasive method is painless, is independent of cardiac function and carries no risk or radiation hazard. The two-dimensional echocardiogram is superior to the standard one-dimensional or M-mode echocardiogram for diagnosis of myxoma in that it can evaluate simultaneously both right and left atria, can detect smaller space-occupying lesions because of better resolution and can more readily determine the size of the lesion and its stalk, can evaluate the mobility of the tumor and can determine more accurately the extent of tumor obstruction. Because of these qualities, we have relied on two-dimensional echocardiography as the definitive diagnostic procedure for detection of atrial myxoma. Currently operation for atrial myxoma is performed without cardiac catheterization or angiocardiography.