We hereby present the case of a 58-year-old male who underwent a total gastrectomy for gastric neoplasm. During the surgery, a tumor mass in the jejunum was identified, considered as metastasis, and resected. The histopathological examination of the jejunal lesion showed ectopic pancreas. In this area, two pathological distinct lesions were identified, one histologically compatible with pancreatic intraepithelial neoplasia (PanIN) type 2 lesion and the other with morphologic criteria for endocrine microadenoma. To our knowledge, this is the first case that evidences the presence of a concomitant premalignant exocrine lesion and benign endocrine lesion in a heterotopic pancreas.