Summary

Christ–Siemens–Touraine syndrome also known as anhidrotic ectodermal dysplasia. Ectodermal dysplasia is a hereditary disorder characterised by developmental dystrophies of ectodermal derivatives. It is characterised by triad of signs comprising sparse hair, abnormal or missing teeth and inability to sweat. Anodontia or hypodontia is the most striking dental manifestation. In severe hypodontia, there is lack of alveolar development with consequent protrusion and eversion of the lips. The case of three children with anhidrotic ectodermal dysplasia with partial anodontia is presented. Dental, oral and physical features were taken into consideration in diagnosis and different treatment modalities for this patient. Clinical management consisted of removable partial prosthesis in maxillary arch and complete denture prosthesis in mandibular arch. The main aim of the treatment was to improve psychological development and to promote better functioning of the stomatognathic system.