Mixed ductal-endocrine carcinoma of the pancreas is composed of ductal and endocrine carcinoma components and each component makes up a significant proportion in the primary tumor as well as in the tumor of metastatic sites. Mixed ductal-endocrine pancreatic carcinoma is exceptionally rare and, to our knowledge, only five cases have been reported in the literature. Recently we experienced a case of mixed ductal-endocrine pancreatic carcinoma with regional lymph node and hepatic metastases in a 63-year-old woman. Here, we report a case of mixed ductal-endocrine pancreatic carcinoma with a review of the literature.