Von Hippel–Lindau (VHL) is an autosomal dominantly inherited familial syndrome characterized by multi-visceral tumors.

Pancreatic lesions of VHL can present as cystic or solid lesions. Most cystic lesions associated with VHL are benign lesions of simple cyst or serous cystadenomas but differentiation of these cystic lesions from premalignant or malignant cystic lesions is essential.

Solid lesions of pancreas are most often neuroendocrine tumors with metastatic potential.

Lesions that are malignant or suspicious of malignancy or causing symptoms of compression require surgical intervention.

Serial cross-sectional imaging studies are recommended for VHL patients as these patients are at higher risk of developing malignant cystic or solid lesions such as cystic neuroendocrine tumors or pancreatic adenocarcinomas.