Children with velocardiofacial syndrome (VCFS) frequently display mathematics difficulties. Research into the specific mathematical strengths and weaknesses in this population is relatively limited. Previous studies into mathematical abilities in VCFS have generally not included an IQ-matched control group. The aim of this study was to assess mathematical ability in children with VCFS, within a cognitive neuropsychological framework, and to compare their performance to a chronologically age- and IQ-matched control group. Twenty-three children with VCFS (aged 8 to 14 years) were administered a range of tasks designed to assess components of McCloskey, Caramazza and Basili’s (1985) cognitive neuropsychological model of mathematics. Children with VCFS did not differ from controls in general mathematical ability, nor did the groups differ on any of the more specific cognitive neuropsychological tasks designed to assess the McCloskey model of mathematics. Results indicate that the weakness in mathematics well-documented in children with VCFS may perhaps reflect overall lowered IQ and associated cognitive dysfunction.