Case 1 was homozygous for both variants, while Case 2 was homozygous for the splice-site variant and heterozygous for the frameshift variant.<h4>Interventions</h4>Both patients received guideline-directed lipid-lowering therapy and ongoing cardiovascular risk management.<h4>Outcomes</h4>Despite biallelic LDLR variants, both patients demonstrated relatively milder hypercholesterolemia and absence of classical HoFH stigmata.<h4>Lessons</h4>The LDLR variants located in exon 18 affecting the cytoplasmic tail domain may be associated with attenuated clinical expression. Here, LDLR is linked to familial hypercholesterolemia.