Severe familial hypercholesterolemia (FH), particularly homozygous FH (HoFH) remains a difficult condition to treat. As a result of markedly elevated LDL-cholesterol levels from conception, subjects with severe FH suffer from accelerated, premature atherosclerotic cardiovascular disease often resulting in premature death. However, over the past three decades there have been remarkable advances in treatment for this condition. Lipid lowering therapies which act mainly by upregulating LDL receptor function, such as high intensity statin, ezetimibe and PCSK9-inhibitors form the backbone of treatment and this combination is often sufficient to attain LDL-cholesterol targets in the majority of subject with heterozygous FH (HeFH). However, a small percentage of HeFH and the vast majority of HoFH patients are unable to achieve acceptable LDL-cholesterol levels with this combination therapy and the addition of therapies that are independent of LDLR function such as lomitapide and more recently the ANGPTL3 inhibitor, evinacumab, are required. These therapies have reduced the need for lipoprotein apheresis which is now only required for the most severe cases, particularly those HoFH patients with minimal or no residual LDLR function. Prospects for gene therapy are promising. However, despite substantial progress, several technical issues still need to be resolved before this therapy can be safely and effectively applied. Overcoming the challenges of severe FH has been a long and difficult journey, but with the treatment options now available, the future for severe FH looks bright.