A 38-year-old woman suffered from syncopes due to torsades de pointes arrhythmias. Echocardiography showed a left ventricular mass that was resected. By immunohistochemical investigations, an organized thrombus was excluded and the diagnosis of a rare ventricular myxoma was confirmed. Postoperatively the arrhythmias resolved without recurrence. We conclude that left ventricular myxoma may cause life-threatening arrhythmias, possibly by irritation of cardiac mechanoreceptors. To our knowledge, this is the first case of a left ventricular myxoma associated with a torsades de pointes arrhythmia.