Although the combination of gastric outlet obstruction and esophageal atresia is rarely seen in neonates, it has been well described. We report the case of a 5-day-old newborn with esophageal atresia and tracheoesophageal fistula associated with complete gastric outlet obstruction due to a mucous plug. As the patient had intense gastric distention, severe respiratory distress requiring ventilatory therapy and complete pyloric obstruction in radiograms, emergency gastrostomy was performed before definitive operation. Definitive treatment consisted of tracheoesophageal fistula ligation and primary esophageal anastomosis. Exploratory laparotomy during the same session revealed a normal pyloric canal, completely obstructed by a firm mucous plug. The plug was removed by pylorotomy, and a pyloroplasty was performed to ease gastric evacuation. Postoperative feeding problems suggested gastric dysmotility as the possible cause for the mucous plug obstruction.