It is recommended that PCSK9 inhibitors be considered in pediatric patients with HoFH but who are not responding adequately to high-intensity statin and ezetimibe therapy; if at least a 15% additional LDL-C reduction is achieved, PCSK9 inhibitor therapy may be continued, but if not, stopping this therapy should be considered [5]. This evidence concerns the gene PCSK9 and homozygous familial hypercholesterolemia.