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Background: Pancreatic heterotopia is a relatively common congenital anomalywhich sometimes becomes symptomatic and mimics other upper gastrointestinal tract (GIT) pathologies.It is the presence of abnormally located pancreatic glandular tissue at sites with no structural or vascularcontact with the main pancreas. It most often occurs in the proximal gastrointestinal tract. The hallmarkof diagnosis is the presence of pancreatic tissue within another, anatomically different organ. CaseReport: We report three patients, I, II, and III, 48, 86, and 33 years of age, respectively, surgicallytreated for symptomatic heterotopic pancreas in the pylorus. A review of the literature on this pathologyis hereby presented. Patients I and II had uneventful postoperative recovery, while patient III developedpostoperative intra-abdominal sepsis due to leakage from the gastric suture line, which was treated withfurther surgery. Histology confirmed pancreatic heterotopia in all cases. All patients made full recoveryand follow-up endoscopy showed no residual disease. Conclusions: Most pancreatic heterotopias are asymptomaticand require no treatment. This entity is extremely difficult to diagnose preoperatively as the causeof upper gastrointestinal tract symptoms and therefore requires a high index of suspicion. Symptomaticlesions should be excised, and this can be safely carried out by minimally invasive techniques dependingon the size and the anatomical location.