Researchers and clinicians have often cited the curious relationship between individuals with Williams syndrome and music. This review aimed to systematically identify, analyze, and synthesize research and findings related to Williams syndrome and music. Thirty-one articles were identified that examined this relationship and were divided into seven areas by their findings. This process covered a diverse array of methodologies, with aims to: 1) report current findings; 2) assess methodological quality; and 3) discuss the potential implications and considerations for the clinical use of music with this population. Collecting and analyzing available research on the relationship between Williams syndrome and music could serve as a platform for future research on the clinical potential for music with this population and guide future inquiry in all areas.