Ectodermal dysplasia is a hereditary disorder characterized by developmental defects of the ectodermal derivatives. It is characterized by aplasia or hypoplasia of structures such as skin, hair, nails, teeth, nerve cells, sweat glands, parts of the eye and ear and other organs. The most prevalent form is hypohydrotic ectodermal dysplasia (HED), also known as Christ–Siemens–Touraine syndrome (CST). This case report describes the manifestations and the dental management of patients with hypohydrotic ectodermal dysplasia. Literature review on ectodermal dysplasia types are also highlighted.