Abstract: Objective　To explore the diagnosis and treatment of noncompaction of ventricular myocardium (NVM) combined with ventricular electrical storm (ES). Methods　The clinical data of a girl with NVM combined with ES for recurrent syncope was retrospectively analyzed, and the relevant literature were reviewed. Results　The female child aged 8 years old was admitted for recurrent syncope within 14 months. Color doppler ultrasonography showed NVM with decreased left ventricle contractile function. Malignant ventricular arrhythmia repeatedly occurred in the form of ES, which was treated with electrocardioversion/ electrodefibrillation, temporary cardiac pacing to maintain hemodynamic stability, and later implantable cardioverter defibrillator (ICD) was implanted. During the one-year follow-up after operation, there was no attack of Adams-Stokes observed, but no significant improvement in cardiac enlargement and cardiac function was reported. Sudden death occurred 14 months after ICD implantation. Conclusion　NVM is a relatively rare type of cardiomyopathy. Ventricular arrhythmia is one of the main clinical manifestations of NVM. Continuous ventricular tachycardia often occurs repeatedly with ES manifestations. Sudden cardiac death may occur.

Key words: noncompaction of ventricular myocardium;　electrical storm;　implantable cardioverter defibrillator; radiofrequency ablation