In FH subjects different mutations in genes encoding for LDL-C metabolism key proteins promote a decreased LDL-C uptake and thus an increased plasma LDL-C concentration [2]; of these, the majority are present in the LDL receptor (LDLR) accounting for 85–90% of cases, while a low prevalence of genetic variants have been reported in apolipoprotein B (ApoB), proprotein convertase sublisin kexin type 9 (PCSK9) or apolipoprotein E (ApoE) [3]. This evidence concerns the gene LDLR and familial hyperaldosteronism.