This chapter reviews the different studies of cognition in velo-cardio-facial syndrome (VCFS), in order to delineate the cognitive phenotype of individuals with VCFS. In order to investigate the underlying basis of the observed impairments as well as areas of relative proficiency in intellectual and academic ability, it is important to use tasks specifically designed to tap into these neuropsychological functions. From the previous reported deficits of nonverbal and mathematical abilities from psychoeducational measures, it is clear that further analysis of spatial cognition in this population is required. Delayed speech and language development is one of the most consistent features in VCFS and a major concern to parents of children with VCFS. The majority of studies of cognition in VCFS have focused on psychoeducational profiles and communicative skills in children with VCFS in order to identify appropriate remedial approaches to the needs of this group.