A case of right ventricular myxoma simulating pulmonary stenosis is reported. The ulti¬mate diagnosis was reached, by echocardiography and magnetic resonance imaging which revealed large filling defects in the right ventricle. The tumor was successfully excised through a right atrial approach, This approach provides adequate surgical-exposure, avoids undue trauma to the ventricular myocardium, and offers an easy way for inspection of the cardiac chambers.