Carmi syndrome, which involves epidermolysis bullosa and pyloric atresia, is a rare neonatal emergency surgical disease. We report Carmi syndrome in a neonate treated surgically. A one-day-old female neonate was consulted due to antenatal polyhydramnios, and no gas was observed on abdominal radiography. There were severe bullous skin lesions on the trunk and areas of aplasia cutis congenita on the neck and extremities. The orogastric tube could not be advanced into the stomach, and it was determined that the opaque contrast material did not pass into the stomach. The patient was opera-ted on with a preliminary diagnosis of esophageal and pyloric atresia. During intraoperative exploration, pyloric atresia was detected. Gastroduode-nostomy and gastrostomy were performed. On the 3rd postoperative day, while the proximal esophagus was aspirated, the orogastric tube reached the stomach. At first, it was thought that the reason why the orogastric tube did not reach the stomach was the narrowing of the his angle following exces-sive gastric dilatation. The patient was started on oral feeding on the 5th postoperative day. Postoperatively, the neonate began to deteriorate and eventually developed septicemia secondary to aplasia cutis and epidermolysis bullosa and passed away on the 24th postnatal day.