The whole-life incidence of PH and PGL is high in familial syndromes with these tumors: 1–5% in neurofibromatosis type 1 (NF1), 15–20% in VHL, 30–50% in multiple endocrine neoplasia type 2 (MEN-2) [2], and probably more than 50% in SDHB and SDHD gene mutation carriers [5,6]. The gene discussed is SDHD; the disease is multiple endocrine neoplasia type 2.