Many transcription factors and other regulatory molecules that drive melanocyte development have been identified through genetic analyses of patients with congenital pigmentation disorders, including piebaldism (SNAI2) [1], Waardenburg syndrome types I and III (PAX3) [2], Waardenburg syndrome type II (SNAI2, MITF, SOX10) [3–5], and Waardenburg-Shah syndrome (EDN3/EDNRB, SOX10) [6,7]. Here, SNAI2 is linked to Waardenburg-Shah syndrome.