BACKGROUND Pancreatic manifestations in Von Hippel-Lindau (VHL) disease can present as a multitude of forms, and their management can be challenging. METHODS A 66-year-old woman presented with increasing abdominal girth without other associated symptoms of nausea, vomiting, abdominal pain, weight-loss, and jaundice. Her medical and surgical histories were significant for type II diabetes, cerebral tumor resection, bilateral nephrectomies, and laser photocoagulation of retinal hemangiomas. Computed tomography (CT) of the abdomen showed a massive multi-cystic lesion in the pancreas and the patient was referred to our hepatopancreatic biliary center. CONCLUSIONS The findings on the subsequent cross-sectional MRI imaging signified pancreatic manifestations in VHL disease. CONCLUSIONS The management of VHL disease-associated benign pancreatic cystic lesions involves interval monitoring with cross-sectional imaging for malignant changes/development.