Bardet-Biedl syndrome (BBS) is a rare autosomal recessive genetic disorder. This syndrome is associated with 19 genes present, and 80% of the cases are determined as a clinical diagnosis result. A 15-year-old female presented with a complaint of gingival bleeding during brushing. As a result of received detailed history and the intraoral-extraoral examinations; retinal dystrophy, obesity, polydactyly, and renal defects of Caucasian female consistent with BBS were reported. Oral and dental findings were high-arched palate, crowding, and missing tooth. As dentists, we should know the diagnostic criteria of this syndrome. In addition, we should have a high index of suspicion so as to enhance the timely recognition of this condition. Dentists should also be familiar with the management protocol that includes a multidisciplinary approach to alleviate the existing conditions.
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