Up to 30% to 40% of adult patients with PHEOs and PGLs (PPGLs), and 80% of pediatric patients, have known susceptibility germline mutations such as multiple endocrine neoplasia (MEN) 2A, MEN 2B, neurofibromatosis 1 (NF1), von Hippel-Lindau disease (VHL), as well as hereditary PPGL syndrome due to mutations of genes encoding for succinate dehydrogenase (SDH) subunits [2-4]. The gene discussed is NF1; the disease is multiple endocrine neoplasia type 2A.