Besides pheochromocytomas, carotid body (CB) paragangliomas and other tumors of neural crest lineage are frequently associated with VHL disease, a hereditary syndrome caused by mutations in the VHL gene and characterized by the occurrence of tumors in multiple tissues (Haase, 2005; Kaelin, 2008; Boedeker et al, 2009). Here, VHL is linked to hereditary pheochromocytoma-paraganglioma.