The first case of LDLR/LDLRAP1 was reported in a cohort of 146 individuals where three Japanese FH patients of the same family were double-heterozygous for a nonsense LDLR p.(Lys790∗) variant and a frameshift p.(Lys204-Glufs∗17) LDLRAP1 variant. The gene discussed is LDLR; the disease is familial hyperaldosteronism.