Up to 20% of patients with pancreatic cancer have a family history of the disease. Familial pancreatic cancer is defined as at least two first-degree relatives with pancreatic cancer that occurs without being part of a cancer syndrome. Although this does not follow a specific Mendelian pattern of inheritance, research is underway to better understand this cohort of patients. Recent expert opinion/recommendations encourage physicians treating patients with pancreatic cancer to ask them to consider having germline testing. Herein, we summarize the current peer-reviewed literature on the most common syndromes associated with pancreatic cancer, associated genetic mutations, and recommendations from various consortia regarding screening strategies. Lastly, we describe the economic impact of pancreatic cancer and the impact of screening tools in high-risk populations.