Any familial hypercholesterolemia in which the cause of the disease is a mutation in the PCSK9 gene. The disease is Monarch Disease Ontology identifier MONDO_0011369 (hypercholesterolemia, autosomal dominant, 3). Also known as: PCSK9 familial hypercholesterolemia, familial hypercholesterolemia caused by mutation in PCSK9, hypercholesterolemia, autosomal dominant, type 3, hypercholesterolemia, familial, 3, low density lipoprotein cholesterol level QTL 1.