We describe our use of intracardiac echocardiography (ICE) to guide endomyocardial biopsy of the left ventricle in a patient with chronic heart failure secondary to endocardial fibroelastosis. Under ICE guidance, we advanced the bioptome through the middle of the mitral annulus, within a trans-septal sheath, and guided the bioptome to the correct location on the mid-posterior wall of the left ventricle. This was performed without adverse events, such as injury to the chordae tendineae or mitral valve regurgitation. Therefore, ICE-guidance is possible for left-sided heart procedures and could lower the risk of complications and improve the biopsy rate for diagnosis.