We present a case of ductal adenocarcinoma originating in a heterotopic pancreas in a 60-year-old patient. The tumor developed at the esophagogastric junction in a hiatal hernia. The nontumoral pancreatic tissue showed ductal cystic dystrophy with enclosed stones, as well as lesions of chronic pancreatitis with metaplastic changes. The perigastric lymph nodes and the liver contained metastatic deposits. Malignant transformation in an ectopic pancreas is exceptional. To our knowledge, this would be the first case occurring in such a location. We review the literature on the subject and discuss the theory of the tumors arising in an aberrant pancreas