Left ventricular noncompaction (LVNC) is a rare congenital disorder of endomyocardial morphogenesis. Since the knowledge of aetiology and pathology is accumulating, the 2006 AHA cardiomyopathy classification sorts LVNC as one of the primary genetic cardiomyopathies. The clinical features of LVNC, however, is not as clear as its aetiology. We summarized the manifestation, hemodynamics, natural course, diagnosis, therapy and prognosis of LVNC by analyzing its clinical features of 2 cases and reviewing the latest related articles.