This paper reviews a series of studies performed to assess the musical abilities and behaviors of individuals with Williams syndrome, a neurogenetic developmental disorder, in the hope of eventually being able to link genes, neurodevelopment, and cognition. Two questionnaire studies addressing the role of music in everyday life, and unusual reactions to sound, are described. Additionally, the findings from two empirical behavioral studies and a neuroimaging study are reviewed. The findings show that individuals with Williams syndrome tend to be more engaged in musical activities than others, and I report a possible neuroanatomical correlate of this engagement, with increased activation in the right amygdala to music and to noise. Williams syndrome represents a compelling model of the relationship between genes, brains, and such complex cognitive behaviors as music.