Following the identification of the von Hippel Lindau (VHL), Neurofibromatosis type (NF1) and Rearranged during transfection (RET) genes responsible for the VHL, NF1 and the multiple endocrine neoplasia type 2 (MEN2) multitumor syndromes respectively in which PPGL is a recurrent feature [22–25], the list has expanded considerably with the advent of comprehensive next-generation sequencing techniques. This evidence concerns the gene NF1 and multiple endocrine neoplasia type 2.