Williams-Beuren syndrome (WBS) is characterized by several diagnostic features, including oligodontia. Restoration of congenitally missing teeth in patients with WBS is a challenge, both emotionally and clinically. In the atrophied alveolar bone process of a juvenile patient with WBS, the maxillary lateral incisors were replaced with two Immediate Provisional Implants (IPI). Early implant loading was used with temporary acrylic resin crowns that were replaced by semi-permanent restorations; the case has been followed for four years. Restoration of the missing maxillary lateral incisors with IPIs and temporary crowns proved to be a convenient, long-lasting and psychologically acceptable solution to the patient and her family.