Abstract: Objective　To assess the clinical features of noncompaction of ventricular myocardium (NVM) in children. Methods  The clinical data from five children with NVM were analyzed, including clinical manifestations, ultrasonic cardiogram(UCG), electrocardiogram, chest X-ray, the process of diagnosis and treatment, and follow up. Results  In five patients (3 boys and 2 girls) aged 3 months to 12 years, four of them were hospitalized because of pneumonia with heart failure. None of the five patients had thromboembolism. Heart enlargement was observed in all five patients on chest X-ray. By 2D ultrasonography, 2 patients had cardiac apex involvement, and 3 patients showed involvement of left ventricular wall and interventricular septum. In one patient, congenital partial defect of the endocardial cushion was observed. All patients were followed up for 3 to 36 months. One patient died of heart failure, 3 patients survived, and one patient was lost to follow-up. Conclusions  The common clinical manifestation of NVM is the heart failure. UCG is preferred for NVM diagnosis. In children with refractory pneumonia and heart failure, NVM should be suspected.