Introduction: Cleft palate is not one of the malformations in the Williams-Beuren syndrome.
Observation: We report the case of a female patient affected by Williams syndrome, presenting with a cleft palate. The diagnosis of Williams-Beuren syndrome was confirmed by genetic analysis.
Discussion: Cleft palate with a Williams syndrome can be fortuitous given these two affections' frequency. The prevalence of Williams syndrome is 1/7500 to 1/10,000 births and palatine division 1/2000 to 1/2500. Cleft palate might also be a rare entity of Williams syndrome. We cannot be sure that the two are linked.