We describe the case of a young patient in whom two-dimensional echocardiography, performed because of episodes of palpitations, discovered the presence of a small mass in the left ventricle, attached to the posterior wall. Transesophageal echocardiography was performed to further assess the mass's attachments and to verify the lack of involvement of the mitral valve and its associated structures. The patient had a cardiopulmonary bypass, surgery and the mass was removed approaching into the left ventricular cavity through the atrial septum via a right atriotomy, without any complication during the postoperative period. In the histologic study the mass appeared to be a typical myxoma. This case has a great interest because the ventricular location of the myxoma is unusual; and because the use of two-dimensional echocardiography, even in the absence of significant symptoms and/or signs, avoided dangerous embolic complications, which often represent the first sign of this neoplastic pathology.