Objectives: The aims of this presentation are to describe the oro-facial findings and the dental management of a patient with a rare genetic disorder, Sotos syndrome.

Methods: Case Description : A ten year old boy was referred to the Department of Paediatic Dentistry, Guys Dental Department for dental care. A detailed medical history revealed that the patient had Sotos syndrome, an atrial ventricular septal defect, asthma and mental disability. Physically the patient was above the 97th percentile for height and weight.

Results: Intra oral examination revealled an early mixed dentition. Gross caries was present in all the primary molars. Extensive caries was also present in the lower left six and upper right six molar teeth. There was also evidence of extensive tooth wear. There was severe lower anterior crowding and the oral hygiene was poor. Radiographic examination confirmed the extent of caries. It also revealed the absence of 18,15,14,12,22,24,25,28,38,35,34,44,45,48.

Management: A preventative programme was implemented for the patient which consisted of oral hygiene instructions , dietary advice and flouride usage.Due to the extent of the dental treatment required and the patient's inability to co-operate it was decided to carry out the patients oral rehabilitation under general anaesthetic and anti-biotic cover.The patient was placed on a three month recall programme to review his dental health and developement.Positive reinforcement of preventative measures are to be under taking at these appointment.

Conclusions: Prompt referral to specalist dental centres is recommended for patients that have craniofacial syndromes . A multi disciplinary approach to the management of these patients is necessary to maximise patient care and outcomes.