We analyzed germline variants in 42 genes (VHL, SDHA, SDHB, SDHC, SDHD, NF1, RET, HRAS, KRAS, EPAS1 (HIF2A), ATRX, CSDE1, BRAF, FGFR1, FGFR2, FGFR3, FGFR4, FGFRL1, SETD2, ARNT, TP53, TP53BP1, TP53BP2, TP53I13, KMT2D, BAP1, IDH1, IDH2, SDHAF1, SDHAP2, FH, EGLN1, MDH2, TMEM127, MAX, KIF1B, MEN1, GDNF, GNAS, CDKN2A, BRCA1, and BRCA2) reported previously to be involved in the development of paragangliomas/pheochromocytomas [34, 52]. This evidence concerns the gene SETD2 and paraganglioma.