We present a rare case of pancreatic adenocarcinoma in a 47-year-old man with the Peutz-Jeghers syndrome. The patient underwent pancreatoduodenectomy with partial resection of the portal vein. We also review the current literature concerning Peutz-Jeghers syndrome associated with malignant tumors, especially pancreatic cancer. To our knowledge, this is the first report of a Peutz-Jeghers syndrome patient with pancreatic cancer having pancreatoduodenectomy and pathologically diagnosed with invasive ductal adenocarcinoma of the pancreas. The Peutz-Jeghers syndrome patients with pancreatic cancer were relatively young. As the pancreatic cancer in these patients was advanced and most were unresectable at diagnosis, the prognoses of these patients were extremely poor. Surgical resection offers the only chance for cure or long-term survival for Peutz-Jeghers syndrome patients, if the tumor is localized without distant metastasis. Therefore, screening even for young patients with Peutz-Jeghers syndrome is necessary for early detection of cancer.