von Hippel-Lindau disease is a hereditary neoplastic syndrome, characterized by malignant and benign lesions in multiple organs. Pancreatic involvement is very common and is in general asymptomatic. We describe a case of malabsorption with severe hypocalcemia in a patient with von Hippel-Lindau disease, caused by exocrine pancreatic insufficiency, probably due to severe cystic transformation of the pancreas.