Double-orifice mitral valve is a rare congenital anomaly. Although it is more frequently associated with other cardiac abnormalities, it may occur as an isolated lesion. There are 2 forms of myocardial noncompaction: isolated and nonisolated myocardial noncompaction. Nonisolated myocardial noncompactions are occasionally reported postnatally in association with congenital heart anomalies such as ventricular septal defect, pulmonic stenosis, and atrial septal defect. To our knowledge, this is the first case presentation reporting a double-orifice mitral valve associated with nonisolated myocardial noncompaction.