Von-Hippel Lindau syndrome (VHL) is a tumor susceptibility syndrome caused by inactivating germline mutations in VHL. Patients with VHL are predisposed to a variety of tumors affecting neural, endocrine, and renal tissues that are exquisitely responsive to hypoxia-dependent signaling; they frequently develop pheochromocytomas. Here, VHL is linked to von Hippel-Lindau disease.