Amelogenesis Imperfecta is a hereditary disorder that affects enamel on primary and permanent teeth. It is a rare dental disease but represents a major restorative challenge for the dentist. A 14-year-old boy presented with sensitive, discolored, and mutilated teeth and a decreased vertical dimension of occlusion. The aim of treatment was to reduce dental sensitivity, to restore esthetics, and to correct the vertical dimension of occlusion. To modify the occlusion, and to protect the dentin from chemical and thermal attacks, nickel-chrome onlays were placed on the molars. To improve the esthetics of the incisors and premolars, resin composite restorations were applied. The patient was regularly recalled during the postoperative period. Radiographic and clinical examinations 10 months posttreatment revealed no evidence of disorders associated with the restored teeth or their supporting structures.