We report here a tricky case of right atrial myxoma with a pulmonary localization mimicking pulmonary thromboembolism. The diagnosis on imaging investigation was delayed because of its atypical appearance. This case report emphasizes the leading role of transthoracic and transesophageal echocardiography in the management of this condition. In autopsy series, the incidence of primary tumors of the heart is evaluated at 0.0017% to 0.19%.(1) Nearly half of them are myxoma.(1, 2) Myxoma are more frequently observed in adults and are commonly localized in the left atrium. Signs and symptoms are comparable to those arising in other cardiovascular and systemic conditions, including variable cardiac murmur, uneasiness, blackout, systemic embolism, cardiac insufficiency, lasting fever, or sudden death.(3) Rare cases of pulmonary embolism have been described. We report here an atypical case of right atrial myxoma with a pulmonary localization mimicking pulmonary embolus.