Congenital esophageal stenosis (CES) is a rare condition. Gross1 reviewed 38 cases of CES from the records of the Boston Children’s Hospital in 1953 and reported that repeated dilatation provided complete relief in most cases; however, he later stated that CES cases who do not respond after six attempts of dilatation should be strongly considered for surgical resection or revision of the strictured area. Since then, a number of treatment modalities have been devised, but there is no consensus regarding which treatment option is the best. Although the efficacy of conservative treatments varies on a case-by-case basis, surgical treatment, which is expected to provide prompt relief of symptoms, carries certain risks of postoperative complications, including leaks, stricture, and gastroesophageal reflux (GER). Surgeons should consider differential diagnoses and understand the pathological basis of CES to be able to employ the most appropriate treatment strategy.