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Non-compaction cardiomyopathy is a recently recognized disorder, based on an arrest in endomyocardial morphogenesis. The disease is characterized by heart failure (both diastolic and systolic), systemic emboli and ventricular arrhythmias. The diagnosis is established by two-dimensional echocardiography. Isolated left ventricular non-compaction cardiomyopathy (IVNC) is an exceedingly rare congenital cardiomyopathy. Only a few cases of this condition have been reported. It is characterized by prominent and excessive trabeculation in a ventricular wall segment, with deep intertrabecular spaces perfused from the ventricular cavity. Echocardiographic findings are important clues for the diagnosis. We report a case of isolated non-compaction of the left ventricular myocardium presented with ventricular tachycardia.