We describe a patient with an unusual segment of ectopic gastric mucosa in the proximal esophagus. The gastric heterotopia was circumferential and unusually long at 7 cm. It contained benign rugal-type folds, a stricture at the mid-portion of the gastric inlet patch was lined by normal antral-type gastric mucosa but harbored submucosally infiltrating adenocarcinoma. There was no evidence of Helicobacter pylori infection by biopsy or serologic screening. Malignancy, including submucosally infiltrating adenocarcinoma, should be considered in patients with strictures involving ectopic gastric mucosa in the proximal esophagus.