Objective To analyze the risk factors of postoperative early death for patients with total anomalous pulmonary venous connection (TAPVC). Methods The clinical data of 52 TAPVC patients without other complex anomalies such as heterotaxy syndrome or single ventricle were collected. The risk factors of postoperative early deaths (patients died within 30 days after surgery) were analyzed. Results Five patients died within 30 d after surgery (9.62%). Among them, 4 patients were less than 4 kg and 1 patient was 4 kg. Their ages were all less than 3 months and 2 were newborns. Two patients were superacardiac type; 1 was cardiac type; and 2 were infracardiac type. Results of T-test and Chi-squared test showed that differences of body weight (P=0.005), body length (P=0.021), anatomical classification (P=0.007), and LA diameter (P=0.016) of the survival group and the death group were statistically significant. The result of binary logistic regression analysis showed that the variable of underweight (≤4 kg) was included in the regression equation (OR=22.857, P=0.009). Conclusion Underweight (≤4 kg) is an independent risk factor of postoperative early death of patients with TAPVC.