ABSTRACT
Multiple endocrine neoplasia syndrome [Type 2B] is a condition associated with systemic malignancies and posses orofacial manifestations. The oral mamifestations help clinician to diagnose disease in early ages. In this article a case is reported with MEN2B syndrome which was diagnosed on the basis of orofacial findings. An eight years old boy was refered for routine dental cares to Mashhad Dental School. In clinical examinations, multiple papules [which defined as neuroma by histopathologic examination] was found on the lip comissures, tip of the tounge and margin of lower eyelids. He had long, narrow face, high arched palate and eversion of eyelids. On the basis of oral and ocular findings, the diagnosis of MEN2B syndrome was made and the patient was refered to a pediatric endocrinologist. Thyroid scan showed cold nodules in thyroid gland which was confirmed by histopathologic examination as medullary carcinoma after total thyroidectomy. Epinephrine and neurepinephrine metabolites were high in urine analysiy setting him in suspicion to develop pheochromocytoma in future. Now the patient is under specialist control. Early diagnosis of MEN2B syndrome is necessary for successful treatment. The dentist may be the first one who diagnoses this syndrome by it's orofacial signs. So Dentists should be aware of oral findings of this disease