The majority of PHEO/PGL cases are known as sporadic tumors while mutations in genes including VHL (von Hippel-Lindau), RET (Multiple Endocrine Neoplasia type 2), NF1 (Neurofibromatosis type 1), SDH (Succinate Dehydrogenase subunits A, B, C and D) and cofactor SDHAF2, MAX (MYC associated factor X), HIF2A (hypoxia-inducible factor 2A), FH (fumarate hydratase) and TMEM127 (transmembrane protein 127) account for approximately 40% of tumors [1]. Here, TMEM127 is linked to neurofibromatosis.