We report a rare case of marked myocardial lipoma of the left ventricle free wall investigated with echocardiography, CT and comprehensive MRI. The case is a 52-year-old man with a history of arrhythmia, hypertension and palpitations. Echocardiography was normal except for the base of the interventricular septum which was slightly thickened. CT and cardiac MRI showed features consistent with myocardial lipoma. This is the first report of myocardial lipoma on the left ventricle free wall depicted as perfusion defect on MR perfusion imaging.