Noncompaction of the ventricular myocardium (NVM) is a rare congenital cardiomyopathy characterized by multiple prominent trabeculations with deep intertrabecular recesses resulting from an arrest in normal embryogenesis of the endocardium and myocardium. The major clinical manifestations are depressed left ventricular systolic and diastolic function, systemic embolism, ventricular tachyarrhythmias, conduction disorders and neurologic abnormalities. We present a 21-year-old female who was diagnosed as dilated cardiomyopathy due to isolated noncompaction of the left ventricle and underwent cardiac transplantation.