Familial hypercholesterolemia is the most prevalent genetic disorder with a autosomal dominant background. Early detection of the condition is important, diagnosing the disease is easily missed in most of the cases until some abnormally early CVD symptoms show up. Statins are the main treatment category to lower the cholesterol levels and control the atherosclerotic complications. Newer therapeutic approaches are developing to more effectively reduce LDL cholesterol levels include, PCSK9 inhibitors, MTP inhibitors, and possibly liver transplantation. lifestyle modification also needs to be taken into account since physical activity and limiting smoking and alcohol intake are very effective in reducing the risk for CVDs.