Incident screen-detected PDAC arising in a low-risk BD-IPMN. A familial pancreatic cancer patient with 3 affected FDR, who at baseline screening had subcentimeter cysts in the head (7 mm, yellow arrow) by MRI (A) and EUS (B). Surveillance continued with annual MRI alternating with EUS. Seven years from baseline, EUS showed a mildly dilated main pancreatic duct (4 mm) in the neck of the gland without a mass and 5 low-risk subcentimeter cystic lesions in the head, body, and tail. These changes did not meet international consensus guideline criteria for resection of IPMN. Repeat EUS at the next scheduled visit 1 year later showed the IPMN in the head (C, yellow arrow) associated with an asymptomatic PDAC (orange arrow, D), not visible on CT. Pancreaticoduodenectomy revealed a moderately differentiated T2N1 PDAC (hematoxylin-eosin stained pathology, image E) arising in an IPMN (F), low-grade IPMN, and extensive low-grade PanIN and associated lobulocentric atrophy (G). The patient is alive and well 4 years later.