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The incidence of esophageal adenocarcinoma is steadily increasing. Most of them, however, are attributable to adenocarcinoma arising from the lower esophagus, which is quite contrary to rare incidence of upper esophageal adenocarcinoma. The origins of esophageal adenocarcinoma are postulated to be Barrett`s esophagus, ectopic gastric mucosa, and esophageal gland. Barrett`s esophagus is widely accepted as being associated with lower esophageal adenocarcinoma. On the other hand, ectopic gastric mucosa, which is believed to remain due to congenital defect during the developmental stage and which is found as many as 10% of routine esophagogastroscopy, has been rarely proven to be the origin of upper esophageal adenocarcinoma. In Korea, to our knowledge, no case has been reported yet, and we herein report a case of upper esophageal adenocarcinoma originated from ectopic gastric mucosa with a brief review of previous reports. The incidence of esophageal adenocarcinoma is steadily increasing. Most of them, however, are attributable to adenocarcinoma arising from the lower esophagus, which is quite contrary to rare incidence of upper esophageal adenocarcinoma. The origins of esophageal adenocarcinoma are postulated to be Barrett`s esophagus, ectopic gastric mucosa, and esophageal gland. Barrett`s esophagus is widely accepted as being associated with lower esophageal adenocarcinoma. On the other hand, ectopic gastric mucosa, which is believed to remain due to congenital defect during the developmental stage and which is found as many as 10% of routine esophagogastroscopy, has been rarely proven to be the origin of upper esophageal adenocarcinoma. In Korea, to our knowledge, no case has been reported yet, and we herein report a case of upper esophageal adenocarcinoma originated from ectopic gastric mucosa with a brief review of previous reports. (Korean J Gastroenterol 2003;41:224-228)