At the turn of the year 2018/19, a new ICD-10 code (E78.0A) will be introduced in Sweden for the hereditary blood lipid disorder familial hypercholesterolemia (FH). Patients with FH have a significantly increased risk of developing atherosclerotic disease, such as myocardial infarction before the age of 50. However, early diagnosis and start of treatment of FH can ameliorate the disease's negative long term effects. The Swedish National Board of Health and Welfare gave in its guidelines from 2015 a high priority to the work of identifying and diagnosing individuals with FH in the general population. The introduction of the ICD-10 code E78.0A for FH may, when properly used, be an effective tool in this work.