Noncompaction of ventricular myocardium (NVM) is a relatively rare congenital disorder characterized by prominent trabeculations and intertrabecular recesses that communicate with the ventricular cavity rather than the coronary circulation.The clinical presentation varies ranging from asymptomatic patients to patients who develop ventricular arrhythmias, thromboembolism, heart failure and sudden cardiac death.We described the case of a 25-year-old man who presented with sudden cardiac arrest.After a series of emergency treatments in Intensive Care Unit and hemodialysis treatment in Nephrology Department, the patient regained consciousness and stable vital signs, and was transferred to Cardiology Department at last.Transthoracic echocardiography showed a trabeculated,sponge-like appearance of both ventricular apical and inferolateral segments.The diagnosis of NVM was made.During medical management, the patient got the sudden appearance of an Adame-Strokes syndrome, when the bedside electrocardiographic monitoring showed a ventricular tachycardia withaheartrateof210beats/min.And the further 24 hours'Holtermonitor showed multifocal ventricular premature beats.Finally an Implantable Cardioverter Defibrillator was planted.Now we discussed the diagnosis of this case and reviewed the medical literature that pertained to NVM.