Abstract Aim The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the survival rate and risk factors of mortality in infants with esophageal atresia (EA). Methods The data of 389 cases registered by 23 centers between 2014 and 2018 were evaluated for demographic features, associated anomalies, surgical treatment and mortality-related outcome. Each parameter was evaluated for cases with mortality (CM) and compared with the results of survivors. Also, prognostic factors were investigated in CM. Results The overall survival rate was 81.09% and the mortality was 19.02% (n = 74). The male/female ratio was 1:1 in CM and 1.2:1 in survivors. The mean weight and height measurements in CM were lower than in survivors (1956 ± 658 g- 43.2 ± 5.7 cm vs 2534 ± 609 g-46.4 ± 4.3 cm) (P < 0.05). The incidence of associated anomalies was 90.6% in CM and significantly higher than survivors (59.6%) (P < 0.05). Neurological, cardiovascular, vertebral, and extremity anomalies were significantly higher in CM (P < 0.001). The most common type of anomaly was EA with distal fistula 89.2% in CM and 74.6% in survivors. The rate of primary anastomosis was also significantly lower in CM (67.6% vs 84.3%, P < 0.05). The gap length did not show statistical difference between CM and survivors (P > 0.05). Tube feeding was more common in CM and the incidence of full oral feedings was significantly higher in survivors (P < 0.01). According to the Spitz classification, the survival rate was 87.1% in Class I, 55.3% in Class II, and 16.7% in Class III. The most common causes of mortality were associated with cardiovascular disease, pneumonia, and sepsis. Conclusion The majority of national TEAR data comes from tertiary healthcare centers in Turkey. Therefore, the slightly increased mortality rates could be related to the inclusion of more complex and complicated cases. Birth weight–height, associated anomalies, and Spitz classification are significant risk factors of mortality in cases with EA.