Fragile X syndrome (FXS) is the main cause of inherited mental retardation and is the result of transcriptional silencing of the fragile X mental retardation gene FMR1. An absence of the associated protein FMRP leads to the deregulation of many genes, which results in phenotypes of Attention-Deficit Hyperactivity Disorder (ADHD), anxiety, epilepsy and autism. The aim of this article is to report the clinical case of twin siblings affected by FXS and to describe the procedures for dental treatment with intravenous sedation. Information regarding the characteristic manifestations of FXS not only aided in the handling of the patients but also enabled us to develop clinical programs to promote and maintain oral health using individualized and specific dental procedures.
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