Cornelia de Lange syndrome (CLS) is a rare genetic disease with an estimated prevalence of 1 in every 30,000 live births. The main features are distinct craniofacial disorders, abnormalities in the upper and lower limbs and intellectual deficit, varying between patients, being the main clinical diagnosis. The aim of the paper is to report a case of a patient with a dental impairment SCL. A 31-year-old male patient with a genetic diagnosis performed at the age of 3 years presented macrodontia, agenesis of upper lateral incisors, impacted teeth, crowding, protrusion of anterior teeth, caries, mandibular micrognathia, anterior open bite, posterior crossbite, palate ogival, periodontal disease and fissured tongue, therefore, he was submitted to dental treatment, under general anesthesia, due to non-collaboration for outpatient treatment. They were performed as tip extractions, restorations with composite resin in units 11 and 21, scaling of sub and supra gingival calculi, as well as topical application of fluoride. The patient remained in the hospital for a day and made a good recovery. The importance of a multidisciplinary team in monitoring patients with SCL, including dentists, should be highlighted, contributing to an improvement in the quality of life of these clients. Patients must have periodic follow-up with the dentist, including in the case reported, will be monitored every 03 months, due to the fact that it is a rare pathology, difficult to manage clinically, for the control and prevention of important dental changes.

The use of a biomodel allows for simulation and aids in team communication, can be used as prostheses, reduces surgical time, and is a reliable copy of bone tissue. Thus, this work aims to report a clinical case of using the biomodel in orthognathic surgery, in order to demonstrate the benefits obtained with the use of biomodels in surgical planning, such as reducing time, increasing predictability, pre-modeling of titanium plates used in bone fixations. Melanoderma patient, male, sought dental care, with dissatisfaction of facial aesthetics and difficulty in phonation, chewing and breathing. After a detailed physical examination and evaluation through imaging tests, Angle skeletal classification III was identified, with indication for surgical treatment. The trans-operative was simplified and faster due to the previous model of the plates and precision of adaptation of them in the bone surface. The use of biomodels and the previous modeling of fixation plates favored greater surgical precision, through the reduction of trans-operative morbidity and reduction of surgical time. Thus, it emphasizes that this type of approach can be an important ally in the treatment of correction of dento-facial deformities. Furthermore, the knowledge of these new tools enabled a better diagnosis and surgical precision.