Lipoprotein(a) and Familial Hypercholesterolemia represent a small group of dyslipidaemias commonly found in patients. Both seem to influence premature atherosclerotic cardiovascular diseases in different ways. Within recent years more and more interest was gained in the exact role of Lipoprotein(a) and also its contribution to the overall cardiovascular risk. In order completely understand the connections it became crucial to understand how Familial Hypercholesterolemia is diagnosed and also treated and how Lipoprotein(a) might influence this process. Also the lack of clear laboratory cut-off values and specific therapies further complicate the general awareness and worsen the outcomes of patients presenting with high Lipoprotein(a) levels. To bring more clearance to this new and evolving field and also evaluate local patient data, the following comprehensive literature review and analysis of a case series was conducted.