Isolated left ventricular noncompaction (IVNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. Clinical presentations of IVNC include systolic and diastolic dysfunction, systemic embolism and ventricular arrhythmias. In recent years there has been an increasing awareness of this anomaly; however, especially in elderly, clinical characterization and natural course of IVNC are still in question. In this case, we report a case of a 78-year-old patient with the diagnosis of IVNC with preserved left ventricular (LV) systolic function. To the best of our knowledge, this is the oldest IVNC case with preserved LV systolic function in the literature.