Hereditary angioedema (HAE) due to C1 Inhibitor (C1-INH) deficiency (C1-INH-HAE) is a rare autosomal dominant disease caused by reduced C1-INH plasma levels (C1-INH-HAE type I) or by the presence of a dysfunctional C1-INH (C1-INH-HAE type II) [1]. Here, SERPING1 is linked to autosomal dominant disease.