With improvements in survival rates in newborns with congenital heart defects (CHDs), focus has now shifted toward enhancing neurodevelopmental outcomes across their life span. To systematically review the prevalence and extent of motor difficulties in infants, children, and adolescents with CHD requiring open-heart surgery. Data sources included Embase, Medline and the Cumulative Index to Nursing and Allied Health Literature. Original studies published between 1997 and 2019 examining gross and/or fine motor skills in children born with a CHD requiring open-heart surgery were selected. The prevalence of motor impairments and mean scores on standardized motor assessments were extracted. Findings were grouped in 5 categories on the basis of the age of the children. Forty-six original studies were included in this systematic review. The prevalence of mild to severe motor impairments (scores <-1 SD below normative data or controls) across childhood ranged from 12.3% to 68.6%, and prevalence ranged from 0% to 60.0% for severe motor impairments (<-2 SDs). Although our results suggest that the overall prevalence of motor impairments <-1 SD remains rather constant across childhood and adolescence, severe motor impairments (<-2 SDs) appear to be more prevalent in younger children. Variability in sampling and methodology between the reviewed studies is the most important limitation of this review. The results of this review highlight that infants with CHD have an increased risk of motor impairments across infancy, childhood, and adolescence. These findings stress the importance of systematic screening or evaluation of motor skills across childhood and adolescence in children with CHD.