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]. The gene discussed is IDH1; the disease is hereditary pheochromocytoma-paraganglioma.