Most PPGLs are sporadic, but 25–40% occur in hereditary syndromes such as multiple endocrine neoplasia type 2 (MEN 2), neurofibromatosis type 1, von Hippel–Lindau syndrome, and hereditary PPGLs due to gene mutations in succinate dehydrogenase (SDHA, SDHAF2, SDHB, C, D), TMEM-127, MAX, FH, EPAS1/HIF-2α, or MDH2 genes [5]. The gene discussed is EPAS1; the disease is von Hippel-Lindau disease.