Left ventricular noncompaction (LVNC) is a disease of myocardium presumably caused by developmental arrest during embryogenesis. LVNC is characterized by the presence of pathological trabeculae in left ventricle. LVNC can be associated with different congenital heart diseases. Different types of myocardium remodeling are follows: dilated, hypertrophic, restrictive and mixed phenotypes. It is unclear whether LVNC is a distinct disease or a morphological feature of different cardiomyopathies. Distinction between pathological trabeculae constituting LVNC and normal phenotype is often difficult. For the time present the considerable attention is paid to the study of disease. Despite the progress in the study of the disease significant controversy remains around understanding of etiology, pathogenesis, clinical characteristics and prognosis of LVNC. Current literature review is aimed to discuss mentioned aspects with a focus on pediatric population.