In the first family (Family 1), we identified an index case of biallelic LDLR variants that included c.1567G > A: p.(Val523Met) and c.344G > A: p.(Arg115His), and in the second family (Family 2), we identified that the wife of the index case’s sibling with FH, who was normolipidemic, had the LDLR c.344G > A: p.(Arg115His) variant. Here, LDLR is linked to familial hyperaldosteronism.