Background Noncompaction of ventricular myocardium (NVM) is a rare type of primary cardiomyopathy.The disease is caused by the disorder in the densification of the myocardium in the early stage of the embryo process.The morphological characteristics are projecting trabeculation in the ventricle and the deep trabecular space interlinked with ventricular chamber.In recent years,many studies have found that the left ventricular growth associated genetic mutation is closely related to the occurrence of NVM.The most clinical manifestations such as heart failure,thromboembolism and arrhythmia are specific.Echocardiography is the most commonly used technique for the diagnosis of NVM.Cardiac computed tomography (CT) scan,cardiac magnetic resonance imaging and left ventricular angiography are other important techniques for its diagnosis.The NVM patients have a long course of disease,poor prognosis and a high rate of misdiagnosis.This article reviews the research progress in the aspects of epidemiological characteristics,genetic characteristics,clinical manifestations,pathophysiology,diagnosis,treatment and so on,in order to provide the basis for the diagnosis and treatment of NVM.