VHL is an autosomal dominant condition with a prevalence of about 1:30,000–40,000 caused by inactivating mutations of the VHL tumor suppressor gene and characterized by the development of hemangioblastomas in the brain, spinal cord, and retina often combined with renal clear cell carcinoma and pheochromocytoma [43]. Here, VHL is linked to pheochromocytoma.