Hypohydrotic ectodermal Dysplasia (Christ-Siemens Touraine syndrome) is a rare genetic disorder that affect several ectodermal structures. The condition is usually inherited as X-linked recessive trait, in which gene is carried by females and manifested in males. The manifestations may vary in individuals and usually involves skin, hair, nail, sweat and sebaceous glands. Hypohydrotic Ectodermal Dysplasia with classical features in two siblings is reported here.