Isolated noncompaction of the ventricular myo-cardium (INVM) is a rare congenital disorder characterized by an arrest of myocardial morphogenesis. We experienced a case of a 23-year-old man, referred to our emergency room, whose symptoms included shortness of breath. ECG-gated cardiac magnetic resonance imaging depicted distinctive features of INVM in both the left and right ventricles. We present the clinical characteristics and morphological features of this case in comparison with some recent reports.