ABSTRACT
@#The most common symptoms of Williams-Beuren syndrome (WBS) are heart defects and unusual facial features. The objective of this case report was to highlight the preparation of comprehensive dental care for this syndrome. A seven-year-old boy came with multiple dental caries as a chief complaint. Parents reported a history of corrected tetralogy of Fallot (ToF) three years ago. The routine medical check-up was carried out annually. Based on the typical signs and symptoms, a diagnosis of William-Beuren Syndrome was made. Comprehensive dental care under general anesthesia on a patient with a history of heart defect and multiple dental caries was the best choice and exhibits a high success rate. Extraction treatment was carried out to avoid the risk of endocarditis infection, restoration to prevent the progressivity of caries, and space maintainers to restore function and aesthetics.