Abstract: Congenital esophageal atresia (EA), a severe malformation of the digestive tract, has an incidence of approximately 1 in every 2500 to 3000 live births. It is characterized by an interruption in the continuity of the esophagus and is often accompanied by tracheoesophageal fistula (TEF) . The etiology of EA is complex. With continuous advancements in prenatal imaging technology, fetal medicine, and neonatal surgery, the prenatal detection rate, perinatal management, and postoperative outcomes of EA have improved. However, the clinical management of EA still faces significant challenges, including, primarily, the inefficiency of prenatal diagnostic techniques in detecting the most common Type C EA, the lack of quantitative decision-making criteria for individualized timing of surgical intervention, and prognostic evaluations that overemphasize short-term anatomical reconstruction while neglecting long-term quality of life. This article systematically reviews the latest research advances in the etiology, prenatal diagnostic strategies, surgical management, and prognostic evaluation of EA. Given the significant improvement in the overall survival rate of pediatric patients with EA, future clinical research and disease management urgently require a paradigm shift—transitioning from a sole focus on surgical anatomical reconstruction to overcoming technical limitations in prenatal diagnosis, optimizing decision-making for individualized surgical timing, and bridging the gap between short-term anatomical reconstruction and long-term quality of life. Furthermore, it is imperative to further strengthen the multidisciplinary collaborative model and establish standardized, whole-life-cycle follow-up and intervention protocols spanning from the prenatal period through adulthood, with the goal of comprehensively improving the long-term physiological and psychological outcomes of these patients.