These tumors occur in the context of several hereditary syndromes, such as Von Hippel Lindau (VHL), Multiple Endocrine Neoplasia type 2, Neurofibromatosis type 1, and the PCC-PGL syndrome, with underlying germline mutations in the VHL, rearranged during transfection (RET), neurofibromin 1 (NF1), and the SDH-genes, respectively [1]. The gene discussed is NF1; the disease is multiple endocrine neoplasia type 2.