Objective To summarize the experience of the superior vena cava and pulmonary connection surgery for functional single ventricle (SV) with total anomalous pulmonary venous (TAPVC). Methods We retrospectively analyzed the clinical data of 10 patients with SV and TAPVC in our hospital from January 2012 through June 2014. There were 7 males and 3 females at average age of 90.33±86.53 months. The 10 patients were with right atrial isomerism, 9 with heterotary and asplenia syndrome. Five patients were anatomic single ventricle and others were with functional uni-ventricle. Nine patients were with supracardiac pattern TAPVC and one was with intracardiac TAPVC. All patients were operated unilateral or bilateral bidirectional Glenn procedure with TAPVC correction. Results The arterial oxygen saturation (SaO2) increased prominently after operation (86%±6% vs. 79%±6%, P<0.01). There were 3 patients with low cardiac output syndrome, one patient with severe arrhythmia, 4 patients with serious pleural effusion, 4 patients with hospital-acquired infection, and 3 patients with central nervous system complications (epilepsy or hemiplegia). One died because of hemorrhage and pulmonary thrombosis, and the other died of hypoxemia and mutiple organ dysfunction syndrome (MODS). Conclusion Glenn is one of palliated procedure choice for SV/TAPVC patients. The indication for surgery and perioperative management individually is crucial.