on Hippel-Lindau (VHL) syndrome is a familial neoplasia syndrome that occurs in approximately one of 36 000 live births (1). The clinical manifestations of VHL include central nervous system hemangioblastomas, retinal hemangiomas, endolymphatic sac tumors, pheochromocytomas, pancreatic tumors and cysts, including pancreatic neuroendocrine tumors (PNETs), and renal cysts and renal cell carcinomas (1). The condition is caused by a germline pathogenic variation in the VHL tumor suppressor gene and is inherited in an autosomal dominant fashion (2).Most of the recommended screening guidelines for pancreas tumors involve nonionizing radiation-emitting imaging modalities (sonography and MRI) instead of CT. If repeated during a lifetime, CT can be associated with substantial radiation exposure (Table 1) (3-8). Both CT (9) and MRI (10) have high sensitivity for the depiction of PNETs, and one report (11) suggested superior sensitivity for intravenous contrast agent-enhanced CT over MRI for the depiction of small PNETs. Hence, some organizations recommend the use of CT in asymptomatic adults (3,12). The frequency of imaging examinations varies in the different guidelines, with some recommending annual screening (1,(4)(5)(6)(7)12,13) and others, biennial evaluations (3,14) depending on whether a pancreatic and/or kidney tumor was already detected in the patient. The medical surveillance of VHL-related PNETs is adapted according to clinical characteristics of the patient and imaging features. MRI is most often used for surveillance of VHL-associated renal cell carcinoma. However, CT is certainly acceptable and may be recommended when needed (3,5,7).The surveillance protocols for patients with VHL are mainly based on several analyses performed in the National Institutes of Health prospective studies and primarily involve repeated CT scans.