Background: This study explores the strategy and effect of emergency surgical treatment for total anomalous pulmonary venous connection (TAPVC). Methods: From March 2009 to February 2020, 78 patients with TAPVC underwent emergency surgical correction. There were 51 males and 27 females. The median age was 39.5 days, and the median weight was 4.0 kg. The preoperative percutaneous oxygen saturation was 80.8±4.5%. Results: Of the cases investigated, seven died during the perioperative period, 16 had delayed chest closure, 19 had early pulmonary vein obstruction, two had secondary tracheal intubation, one had a brain complication, and one had third-degree atrioventricular block. Low weight, younger age, cardiopulmonary bypass time, and aortic cross-clamp time were identified as risk factors for early mortality. During the follow-up from four to 137 months, 12 cases did not respond to follow up. Ten patients died within one to six months after discharge. One patient underwent reoperation due to pulmonary vein obstruction. The longer hospital stays after operation and intensive care unit time were identified as risk factors for late mortality. Conclusions: Emergency surgery for severe TAPVC patients after admission had achieved good results in the near future. Prenatal diagnosis should be strengthened to save more patients. The higher late mortality rate indicates that such patients should strengthen post-discharge management to reduce the occurrence of post-discharge deaths.