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Background The surgical outcomes have been greatly improved over the past four decades for functional sin-gle ventricle(FSV). However,the repair for coexistence of FSV and total anomalous pulmonary venous connec-tion(TAPVC),especially obstructed TAPVC,has remained a clinical challenge. Little is known about the re-sults of surgical treatment for patients with FSV-TAPVC in China. Methods Forty consecutive patients with FSV-TAPVC undergoing initial surgical palliation(median age:16.8 months;body weight:8.25 kg)were ret-rospectively enrolled from 2006 to 2016 in a specialized cardiovascular institute. TAPVC was repaired in 30 pa-tients. The mean follow-up period was 49 months. Results All of the 7 patients with preoperative pulmonary vein obstruction(PVO)underwent repair of TAPVC at the first operation and survived. When a systemic to pul-monary(S-P)shunt or pulmonary artery banding(PAB)was necessary,patients with concomitant TAPVC re-pair had lower mortality (30.0％) and higher total cavopulmonary connection completion rate (20.0％) than those without TAPVC repair[75.0％ and 0％,respectively],although with no statistical significance(Fisher test,P=0.505 and P=0.245). The overall survival rates for the cohort at 1,3 and 5 years after the initial surgical intervention were 80.0％,77.1％and 77.1％,respectively. Multivariate COX regression analysis detected that S-P shunt(adjusted odds ratio:6.51;95％CI:1.26-33.71,P=0.030)is the only risk factor for survival. The rein-tervention rate for postoperative PVO was higher in patients with preoperative PVO than those without(57.1％vs. 4.3％,Log Rank:P=0.006). Conclusion The mid-term results of surgical repair of FSV-TAPVC are still challenging. When PVO exists,surgical repair for TAPVC is suggested as soon as possible. Repair for TAPVC should also be considered when S-P shunt or PAB is necessary. Further study with larger population are warrant-ed to support our finding.