n contrast to many other malignant entities the risk factors for pancreatic cancer are mostly unknown. Hereditary factors are causative in about 3–6% of all pancreatic cancers. In addition to hereditary tumor disposition syndromes and hereditary pancreatitis, the familial pancreatic cancer syndrome accounts for about 75% of all hereditary pancreatic cancers. A familial pancreatic cancer syndrome is present when at least two first-degree relatives in a family have histologically proven pancreatic cancer and there are no criteria for another hereditary syndrome. An underlying uniform monogenetic gene defect in familial pancreatic cancer syndrome is currently unknown and a germline mutation in the BRCA2 gene is so far the most frequently identified genetic alteration. Patients at risk for familial pancreatic cancer should be offered participation in registry studies with associated early detection programs (e.g., the FaPaCa registry). These enable the detection of high-grade neoplasms and pancreatic cancer in the early stages, which appears to reduce mortality; however, the detected pancreatic lesions are often incorrectly diagnostically classified, and patients undergo complex pancreatic resection despite benign findings. Finally, studies have so far not been able to clearly clarify whether patients at risk benefit from an early detection program. According to current data, prophylactic operations are not indicated for familial pancreatic cancer syndrome and should only be performed if there is evidence of a suspicious finding that requires resection.