Left ventricular noncompaction (LVNC) is a rare cardiomyopathy. It usually presents with ventricular dysfunction, thromboembolic events and arrhythmias. An asymptomatic clinical course is also possible. LVNC is frequently associated with other congenital heart diseases including heart valve abnormalities. The coexistence of LVNC with double-orifice mitral valve was observed rarely. The presence of such coexistence in a patient presented with heart failure in the newborn period is reported herein because of its rarity.