This study is the first to report an association between TS and 22q11DupS, thereby broadening the link between copy number variation at 22q11.2 and the development of TS and comorbid neurobehavioral disorders including OCB. Further research should delineate the basis for linkage between one or more genes within the 22q11.2 ∼3 Mb critical recombination region and neuropsychiatric disorders.