To date, numerous pancreatic lesions have been reported in patients with VHL, including serous cyst neoplasia (SCN) [4], pancreatic-neuroendocrine tumor (p-NET) [5,6], adenocarcinomas [7], hemangioblastomas [1], metastasis of renal cell carcinoma [8] and MSNN [9]. This evidence concerns the gene VHL and hemangioblastoma.