Orthodontic treatment in a young patient with Beckwith-Wiedeman Syndrome (BWS) is reported and a multidisciplinary approach to the management of this syndrome is reviewed. The patient presented with a tendency to Class III malocclusion, an open-bite and a slight macroglossia, which was treated at an early age by glossotomy. It was decided to monitor growth without treatment and to wait for the best time to begin therapy. It was based only on functional rehabilitation, without any fixed appliances, in which optimum intercuspation of the teeth and the skeletal Class I relationship was achieved and maintained after the retention period of three years. In conclusion the treatment of BWS patients requires a multidisciplinary approach that includes orthodontics, orthopaedics and surgical intervention. It is also necessary to underline the significance of diagnosis at an early age and timely treatment to reduce the development of dento-skeletal alterations.