The incidence is 2–8/1,000,000 per year1, 2, and up to 40% of all PPGL are known to be part of inherited syndromes, including Neurofibromatosis type 1, Multiple Endocrine Neoplasia type 2A and 2B (MEN2A/MEN2B) as well as von Hippel-Lindau syndrome3. Here, RET is linked to neurofibromatosis type 1.