The term 'familial pancreatic cancer (FPC) defines families with at least two first-degree relatives with confirmed pancreatic cancer that do not fulfill the criteria of other inherited cancer syndromes with an increased risk for the development of PC, such as Peutz-Jeghers syndrome, hereditary pancreatitis and hereditary breast and ovarian cancer. FPC is a mostly autosomal dominant inherited tumor syndrome with a heterogeneous phenotype. The major genetic defect has not been identified yet, although mutations, for example in the BRCA2 gene, could be identified in some FPC families. Nevertheless, most experts recommend participation in screening and surveillance programs to high-risk individuals. Most board-approved screening programs are based on endoscopic ultrasound. The first data on the prospective screening of high-risk individuals have demonstrated that precursor lesions of PC can be identified, but false-positive findings do occur.