Williams syndrome is a rare genetic condition, which is caused by the deletion of genetic material from the region q11.23 of chromosome 7. It is characterized by multiple congenital anomalies, including distinctive facial appearance, cardiovascular anomalies, mental retardation, and a unique personality with high levels of empathy and anxiety A 6 year old Asian girl with Williams Syndrome came to Taipei Medical University Hospital for dental treatment. The main difficulties for this girl were her cognitive problems. She did not have other major systemic problems such as cardiovascular disease. She had caries in multiple teeth and required extensive dental treatment. She received the pulp treatment, the extraction of primary teeth, stainless-steel crown restorations, sealants and fluoride application as preventive measures. In this case, the 6-year-old girl received care with the use of a papoose board on several occasions. The papoose board use was coupled with successful behavioral training which has allowed her to receive dental care without the board for many dental procedures.