While patients with VHL disease may develop various types of tumors, including clear cell renal cell carcinomas (RCC), pancreatic neuroendocrine tumors (pNETs), pheochromocytomas and paragangliomas, and endolymphatic sac tumors, central nervous system hemangioblastoma (CNS-Hb) is typically the first manifestation of VHL disease [2], occurring in 70-80% of cases [8]. The gene discussed is GSTM1; the disease is pancreatic neuroendocrine tumor.