Backgroud: Surgical results of functional single ventricle (FSV) patients with totally anomalous pulmonary venous connection (TAPVC), have a poor outcome. We retrospectively analyzed our 10-year surgical clinical experience and risk factors of mortality of these patients. Methods: Between March 2008 and August 2018, 43 consecutive patients with FSV and TAPVC underwent initial surgical palliation and TAPVC repair or not. The median body weight and age were 12 (range 5-44) kg and 32 (range 2-256) months, respectively. Among these cases, there are 19 cases of supracardiac TAPVC, 22 of intracardiac type, and 2 of mixed type. 12 patients need to perform TAPVC repair during initial surgical palliation (supracardiac in 10 and mixed type in 2). Results: Overall survival at 1 and 5 years were 89.5% and 83.3%, respectively. In TAPVC repair group and non-TAPVC repair group, overall survival after the initial surgical palliation were 58.3 and 87.1% at 1 year, 40.0% and 87.1% at 3 years, respectively. Cox univariate analysis detected that preoperative pulmonary vein obstruction (PVO) (p=0.047) and concomitant TAPVC repair (p=0.007) were risk factors for mortality, and multivariable analysis indicated concomitant TAPVC repair as the only factor (p=0.033). Conclusions: The mid-term results of surgical results of FSV associated with TAPVC, especially for patients who need to concomitant TAPVC repair, remain poor. Preoperative PVO is identified as risk factor that increases mortality of these patients.