Recently, Bork et al. [9] reported a hitherto unknown variant in exon 10 of the KNG1 gene (c.1136T>A, p.Met379Lys) co-segregated with clinical symptoms of hereditary angioedema (HAE) with normal C1-INH levels in three generations of a large German family. Here, KNG1 is linked to hereditary angioedema.