Aims: To determine the motor and cognitive outcome in a regional cohort of survivors of surgically corrected congenital diaphragmatic hernia. Methods: Thirty-three children (85% of survivors) were examined at a mean age of 8.6 years (3.3-15.7 years), seven had a genetic comorbidity. Outcome was assessed with the Wechsler Preschool and Primary Scale of Intelligence 3rd version and the Wechsler Intelligence Scale 4th version. Motor performance was tested with the Movement Assessment Battery for Children 2nd edition in children younger than 5 years and thereafter with the Zurich Neuromotor Assessment. Results: Children without a genetic comorbidity had a normal cognitive outcome (median IQ 103, 70-121), but fine motor (p = 0.008) and gross motor outcome (p = 0.001) were poorer than the norm. Medical variables were not predictive of adverse outcome, whereas genetic comorbidity was the strongest predictor of low IQ (p < 0.001) and of poor motor performance (p = 0.04). Conclusions: In the absence of a genetic comorbidity, children with congenital diaphragmatic hernia have a favourable cognitive outcome, but motor outcome may be affected. This needs to be taken into account for parental counselling. Further, long-term neurodevelopmental assessment in children with congenital diaphragmatic hernia is important to provide early therapeutic interventions.