The aim of this study was to compare the motor development of primary school children (age 5-14y) with a 22q11 deletion (del22q11) group and a control group. The effects of a congenital heart defect (CHD) and IQ on motor development were additionally studied within the del22q11 group. Motor development of 37 children with a del22q11 (20 males, 17 females; mean age 9y 4mo, range 5y 9mo-13y 3mo) and 34 controls (23 males, 11 females; mean age 9y 1mo, range 4y 8mo-13y 6mo) was assessed with the Bruininks-Oseretsky Test of Motor Proficiency. The del22q11 group showed a significant deficit in motor functioning compared with the control group (p < 0.01). Within the del22q11 group there was a significant effect of IQ on motor performance, but no effect of CHD was found. To conclude, primary school children with a del22q11 syndrome showed a significant deficit in motor performance compared with a control group. A significant effect of IQ on motor performance in del22q11 was found.