A case of amelogenesis imperfecta with hypoplasia, hypocalcification of the enamel, congenital absence of teeth 12 and 22, delayed eruption of tooth 23, edge-to-edge incisal relationship, open bite, and bilateral posterior cross bite at the level of the first and second premolars is presented. Lateral skull teleradiography indicated a Class III skeletal pattern of maxillary origin associated with a dolichofacial pattern with multiple indicators of facial hyperdivergence. The patient presented a major esthetic abnormality of the face and required orthodontic treatment prior to a prosthetic solution with full-coverage metal-ceramic crowns in both the maxilla and the mandible. The diagnosis of cases such as this one and the therapeutic implications from an orthodontic and prosthetic standpoint are reported.