Isolated right ventricular noncompaction (IRNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The clinical syndrome includes systolic and diastolic dysfunction; some cases may have ventricular arrhythmias. We report a case of a female with the diagnosis of right ventricular non-compaction myocardium (RVNC) with normal left ventricular systolic function. To the best of our knowledge, there have been no reports of isolated ventricular non-compaction involving only the right ventricular before 2008, and there have only been described in very few cases of newborns and adult patients.