Dental rehabilitation of acute cases of enamel renal syndrome is challenging due to the absence of clinical reports. In the present case history report, examination of an 18-year-old patient revealed a complete lack of permanent teeth, as well as irregular and swollen bone and gingival morphology. Radiographs showed multiple impacted teeth in both arches. Creating a 1.5- to 2-cm interarch space was necessary for setting complete dentures. The ideal occlusal plane was chosen by combining two techniques (cephalometric radiograph and modification of the mandibular occlusal rim according to anatomical guidelines). Extraction of impacted teeth and recontouring of the alveolar process were performed simultaneously. The mandibular denture was connected through Locator abutments to two symphyseal implants. This pioneering clinical report will provide guidance to practitioners in the surgical intervention of patients with FAM20A (family with sequence similarities 20 A) gene mutations.