Ectodermal dysplasia (ED) is a hereditary disorder characterized by abnormal development of certain tissues and structures of ectodermal origin, like the sweat glands, scalp hair, nails, skin pigmentation, and craniofacial structures. Orofacial characteristics of this syndrome include anodontia or hypodontia, hypoplastic conical teeth, underdevelopment of the alveolar ridges, frontal bossing, a depressed nasal bridge, protuberant lips, and hypotrichosis. A 12-year-old male patient exhibiting the classical features of HED (hypohidrosis, hypotrichosis and hypodontia), visited our Department of Prosthodontics at Chatrapati Shahu Maharaj Shikshan Sanstha Dental College and Hospital . In the clinical and radiographic evaluation, it was evident that the only erupted teeth were three conical teeth in the anterior region and one posterior tooth in the left side of the maxillary arch. Resin composite restoration of occlusal morphology of anterior conical teeth followed by provisional removable partial dentures in the maxillary arch and complete denture in the mandibular arch was fabricated for prosthodontic rehabilitation of this patient after considering his growth and the number and condition of his present teeth. At the 6-month follow-up no major complications occurred and definitive treatment can be planned (if necessary) after the completion of growth.
