Objective：To investigate the clinical features and therapeutic effects of noncompaction of ventricular myocardium（NVM） in children. Methods：From September 2007 to November 2012，20 cases of NVM admitted in the Children’s Hospital of Chongqing Medical University were retrospectively analyzed. The retrospective analysis was carried out according to clinical features，auxiliary examinations（chest X-ray，electrocardiogram（ECG），ultrasonic cardiogram（UCG） and cardiac magnetic resonance（CMR），etc），clinical treatments and follow-up results，etc. Results：Among the 20 cases，2 cases had family history，16 cases（80%） showed cardiac insufficiency，10 cases（50%） demonstrated arrhythmia，and thrombosis or embolic events did not occur during this time. Chest X-ray of 12 cases（60%） showed cardiomegaly. Typical changes of NVM were seen in UCG of all patients：17 cases had only left ventricular pathological changes，2 cases had double ventricular pathological changes，9 cases（45%） had combination of congenital heart disease，3 cases（15%） had combination of dilated cardiomyopathy（DCM），4 cases（20%） had combination of endocardial fibroelastosis（EFE）. After all 20 children being treated in the hospital for the first time，16 children were cured and discharged from the hospital and 4 children gave up the treatment. The average follow-up time of the 20 patients was 7.86 months（1-15 months）. Five children died of the aggravation of heart failure. During the follow-up，symptoms of 8 children were significantly alleviated and modified Ross score was lower than that before treatment. Regular UCG recheck showed that the cardiac function was improved well and ejection fraction of left ventricular can be seen by UCG successfully after the treatment，with statistic significances（t=-3.248，P=0.018）. No statistical difference in shortening fraction of left ventricular，left ven－tricular end diastolic diameter，right ventricular end diastolic diameter and ratio of noncompacted to compacted myocardial layers after the treatment was observed（t=-1.348，P＝０.２２６；t=0.343，P=0.744；t=0.969，P=0.370；t=0.467，P=0.657）. Conclusion：Cardiac insuffi－ciency with different degrees is the chief complaint of NVM patients. The main auxiliary examination for the diagnosis of NVM is color Doppler electrocardiography. With active internal medicine treatment，clinical symptoms and left ventricular dysfunction can be greatly improved in short-term，but the long-term follow-up shows a poor prognosis in conclusion.