RESUMO
Intestinal duplications located in the duodenum are rare conditions that generally affect the first or second duodenal portion. It is extremely unusual for this condition to be located in the pyloroduodenal area, accounting for less than ten documented cases. This research presents a case of a female newborn who showed on the second day of life signs of upper intestinal obstruction which required exploratory laparotomy. The surgery revealed a pyloroduodenal cyst that was successfully excised. This is an extremely rare congenital anomaly that encompasses challenging symptoms and signs, and the purpose of the surgery should be the complete resection of the lesion. If the complete excision endangers the surrounding organs, partial resection through mucosectomy is a valid alternative to prevent the complications of more invasive surgery.