The purpose of this study was to explore the relationship between intelligence and visual motor integration skills in 5-year-old children with 22q11-deletion syndrome (22q11DS) (N = 65, 43 females, 22 males; mean age 5.6 years (SD 0.2), range 5.23-5.99 years). Sufficient VMI skills seem a prerequisite for IQ testing. Since problems related to these skills are reported in children with 22q11DS, weak VMI skills may contribute to the lower than average IQ scores commonly reported. To investigate if the correlation of VMI and IQ score was mainly influenced by problems with visual perception skills (VP), motor coordination skills (MC) or difficulties with the integration of both skills (VMI), a subgroup (n = 28) was also administered the "Beery VMI" supplemental developmental tests. Due to the narrow age range of this study, we were also able to provide an insight into the neurocognitive phenotype of 5-year olds with 22q11DS and the influence of gender, heart disease and origin of deletion on this phenotype. Results show a mean full scale IQ (FSIQ) = 73.0 (SD 10.4) and mean VMI = 86.2 (SD 8.4). A significant correlation between FSIQ and VMI was found (r = 0.45, p = 0.000), with most variation (26%) explained in the performance IQ score ((PIQ), r = 0.51, p = 0.000). VP correlated significantly with FSIQ (r = 0.44, p = 0.01) and PIQ (r = 0.49, p = 0.004). MC was not significantly correlated with IQ (FSIQ, r = 0.21, p = 0.15; PIQ, r = 0.28, p = 0.07), suggesting that problems with motor coordination do not influence results on IQ-tests in a significant way at this age. Girls scored significantly higher on FSIQ and PIQ than boys; cardiac anomalies were not predictive of FSIQ or VMI scores. The results of this study suggest a characteristic neurocognitive phenotype for 5-year olds with 22q11DS. Deficiencies in visual perception and/or processing are negatively correlated with IQ scores, whereas deficiencies in motor skills do not have a relevant negative impact at this age. These findings provide further insight into 22q11DS specific neurocognitive deficiencies. (Contains 1 figure and 3 tables.)