This report describes a rare pediatric case of compound heterozygous FH (cHeFH), in which two mutations were identified in the low-density lipoprotein receptor (<i>LDLR</i>) gene through whole-exome sequencing (WES), including c.682G > A and the rarely reported c.1187-10G > A variant. The gene discussed is LDLR; the disease is familial hyperaldosteronism.