Today, the majority of PCC/PGLs are diagnosed incidentally during unrelated examinations, followed by cases presenting with symptoms of catecholamine excess, and thirdly, during screenings of patients with known familial syndromes (such as MEN2, von Hippel-Lindau syndrome, neurofibromatosis type 1, or mutations in genes like succinate dehydrogenase [SDH], TMEM-127, MAX, FH, EPAS1/HIF-2α, or MDH2) [2]. The gene discussed is EPAS1; the disease is von Hippel-Lindau disease.