Abstract: Hypohidrosis ectodermal dysplasia（HED） is a rare congenital disorder that results in abnormalities in the structures of ectodermal origin tissues, such as teeth, eccrine sweat glands, hair and nails. However, most of studys in HED is presented in the form of clinic case reports. This article reviews the research progress of HED in types, genetic locations, proteins, and signaling pathways in odontogenesis.