Esophageal atresia is a congenital anomaly that is considered as the absence of esophageal lumen, often associated with tracheo-esophageal fistula. It appears in about 1 out of 3,000 live-born children. Etiology is not fully clarified, but it is presumed to be a consequence of the developmental disorders of the esophagus from the 4th to 6th embryonic week. There are 6 types of atresia, and the most common is esophageal atresia with distal tracheo-esophageal fistula. The suspicion is based on ultrasound examination of the fetus - a finding of polyhydramniosis or hypoplasia of the stomach. After birth there is hypersalination and choking, coughing and regurgitation during feeding, and in the presence of fistula there are frequent pneumonia. The diagnosis is based on the symptoms and is confirmed by the stifling the catheter in the esophageal pouch and by the X-ray diagnostics. An urgent surgical procedure is needed, and the aim is to close the trachea-esophageal fistula and to establish the continuity of the digestive tract. If the distance between the upper and lower pouch is short, the immediate primary anastomosis is established. In cases of the long gap between the upper and lower pouch, it is necessary to bridge the gap between pouches or to reconstruct the esophagus using an intestinal segment, the gastric lobe or the gastric tube. The risk is higher in children with low birth weight, with associative malformation and respiratory disease, and delayed and staged repair is recommended. Postoperative early and late complications are possible. The prognosis depends on the condition of the child and also on preoperative care, selecting the most appropriate surgical procedure and the postoperative complications.