A case of adenomyoma of the prepyloric antrum and a review of the previous reported in the literature are presented. The tumor is composed of cysts and glandular structures lined by cuboidal-to-columnar epithelium surrounded by hypertrophic smooth muscle bundles. Furthermore, glands resembling Brunner's and/or heterotopic pancreatic tissue are present in some patients. The endoscopic characteristics of the lesion are discussed, as well as the diagnostic and therapeutic approaches.