PURPOSE: The purpose of this study is to determine the feasibility of biventricular repair for hypoplastic left ventricle combined with coarctation of aorta and to provide strategy of surgical treatment. METHODS: The preoperative and postoperative dimension of mitral valve and left ventricle was compared for three patients with hypoplastic left ventricle combined with coarctation of aorta. Ventricular septal defect, atrial septal defect and patent ductus arteriosus were also present in all patients, and the age at the operation was 36, 15 and 11 days. The preoperative end-diastolic left ventricular volume was 13, 28, 24 mL/m2 respectively, and antegrade flow was observed in ascending aorta in all patients. RESULTS: All patients underwent repair of coarctation of aorta. In addition to coarctation repair, pulmonary arterial banding and atrial septal defect closure was performed in Case 1 and 2 respectively, leaving the ventricular septal defect unclosed. In case 3, the ventricular septal defect was closed with the coarctation repair. Case 1 had to undergo Norwood procedure due to inadequate growth of left ventricle and is waiting for univentricular repair. In Case 2 and 3, the left ventricular size increased and biventricular repair could be performed successfully. CONCLUSION: Left ventricular growth can be obtained by repair of coarctation and biventricular repair was feasible in selected cases of hypoplastic left ventricle combined with coarctation of aorta. Size and volume measurement of mitral valve and left ventricle with echocardiography or magnetic resonance image provide useful information to decide the surgical strategy for this group of patients.