In Carney‐Stratakis syndrome, SDH deficiency is caused by germline mutations in SDHB, SDHC, or SDHD. 10, 14, 15 However, the Carney triad and other pediatric cases are most commonly caused by epigenetic silencing of the SDHC gene through promoter hypermethylation.10, 16 Very recently, Benn et al. found that pathogenic SDHA‐C variants present as germline events in the general population with tumors not driven by these mutations in up to 25.6%.17 The authors used a Bayesian approach to calculate penetrance for SDHA variants at 1.7% (95% CI 0.8% to 3.8%). This evidence concerns the gene SDHB and hyperinsulinemic hypoglycemia, familial, 4.