BACKGROUND The international Cancer of the Pancreas Screening (CAPS) Consortium has recommended specific guidelines for the management of individuals with increased risk of pancreatic cancer (high-risk individuals; HRI) arising from a familial clustering of the disease or associated with germline mutations. OBJECTIVE To update the 2013 CAPS consensus guidelines on the management of HRI. METHODS A modified Delphi approach was used, consisting of a systematic review of the literature, an international multidisciplinary development workgroup meeting, and two rounds of online voting. Based on pre-defined criteria, experts in the field of familial pancreatic cancer were invited to vote on statements using a 7-point Likert scale. An a priori threshold of 75% agreement (‘Strongly agree’ or ‘Agree‘) was used to establish consensus statements. RESULTS 76 experts, from 7 disciplines, 11 countries and 4 continents, completed two rounds of voting (response rate 84%). Consensus was reached on more statements than the previous guidelines (55 versus 34). The goals of surveillance (to identify T1N0M0 margin-negative pancreatic cancer and high-grade dysplastic precursor lesions) remained unchanged. Experts now also agreed that surveillance should commence at least by the age of 50 or 10 years younger than onset in the family, or when diabetes develops. There was still no agreement on the age to stop surveillance. Added as eligible for surveillance were CDKN2A p16 mutation carriers without a pancreatic cancer family history, and ATM mutation carriers with one affected first-degree relative. Experts also agreed that baseline surveillance should still include both endoscopic ultrasound (EUS) and MRI/MRCP, and not CT, ERCP, or abdominal ultrasound. Both modalities should also be used for follow-up, but there was no consensus on whether to alternate these modalities, or on the optimal surveillance intervals when lesions are detected. Serum carbohydrate antigen 19-9 was recommended when worrisome features are found on imaging. Fasting blood glucose testing should be performed routinely, and a new diagnosis of diabetes should prompt for immediate investigations. The surveillance interval in case of no or low-risk findings should be 12 months. EUS-fine needle aspiration is recommended for detected cysts with worrisome features, solid lesions ≥5 mm, or main pancreatic duct strictures (with or without associated mass). Main areas of disagreement included if and how surveillance should be performed for hereditary pancreatitis, the management of indeterminate lesions, and the type of surgery. CONCLUSION Surveillance is recommended for selected HRI to detect early pancreatic cancer and its high-grade precursors, and should be performed in expertise centers, by multidisciplinary teams, preferably within a research setting.