The purpose of this study was to define the role and indications of partial left ventriculectomy (PLV) in children with end-stage dilated cardiomyopathy (DCM). Clinical data were collected by retrospective chart review of children with DCM who were treated from 1997 to 2000. Four patients underwent PLV (PLV group) and 5 patients were managed without PLV (non-PLV group). In the PLV group, 2 patients are well 18 and 35 months postoperatively. One infant survived 6 months and then successfully underwent heart transplantation, and the other child died of hemoptysis 2 weeks postoperatively. Factors affecting outcome were preoperative status, in particular whether surgery was performed urgently or electively. In the non-PLV group, 4 patients were well controlled by medical treatment and 1 infant underwent mitral valve replacement for severe mitral regurgitation. The cardiothoracic ratio ranged from 72% to 76% in the PLV group and from 45% to 60% in the non-PLV group. The percentage of the expected left ventricular diastolic dimension ranged from 184% to 218% in the PLV group and from 109% to 163% in the non-PLV group. Ejection fractions in the PLV group were from 10% to 22% and from 36% to 56% in the non-PLV group. The serum brain natriuretic peptide concentration was above 1,200 pg/ml in the PLV group and below 168 pg/ml in the non-PLV group. In conclusion, PLV is indicated for selected children with end-stage DCM, and is most appropriate when medical therapy is not effective and heart transplantation is unavailable.