Background: In the literature, pancreatic cancer is not frequently acknowledged among the tumors that are considered a part of Lynch Syndrome. Case Presentation: Our case is one of a young man who was found, very early in life, to have pancreatic cancer. His tumor demonstrated germline microsatellite instability, and hence by definition the patient has Lynch syndrome. He responded well to treatment, which included surgery and adjuvant chemotherapy. To date he remains in remission from pancreatic cancer. Conclusion: The rare instances in this case report include: (a) The patient had pancreatic cancer that fulfilled the histopathological and clinical criteria for Lynch syndrome. (b) Pancreatic cancer was diagnosed earlier in our patient than is expected in patients who suffer from pancreatic cancer as a part of Lynch syndrome. (c) Our patient had an excellent response to chemotherapy. He remains in remission to date from pancreatic cancer and is 5 years since his last treatment for this disease.