Von Hippel-Lindau disease is an autosomal dominant disorder characterized by the presence of multiple benign and malignant tumors including hemangioblastomas of the retina and central nervous system, pancreatic cysts and tumors, renal cell carcinomas, pheochromocytomas and epididymal cystadenomas. We report the radiologic findings of a case of von Hippel-Lindau disease, describing the family history.