Cardiac myxomas are the most common primary benign tumors of the heart and appears particularly in the left atrium. Myxomas exhibit a wide spectrum of symptoms from asymptomatic to very serious according to the localisation of the tumours. Only 5% of these tumours occur in the right and left ventricles. We report an unusual case of a 21-year-old woman with a leukemia in remission, in whom routine transthorasic echocardiographic examination demonstrated an incidental left ventricular mass. The patient underwent successful resection of the left ventricular mass under cardiopulmonary bypass through the left atrial approach. Histopathologic examination was diagnostic for myxoma. In this case, the presentation of the mass was atypical for two reasons: the tumour was a cardiac myxoma in the left ventricular localisation and in a patient with leukemia in remission.