Objectives Grip strength is known to be reduced in adults with congenital heart disease (CHD). This study compared grip strength in paediatric patients with CHD with healthy controls and determined a possible association between grip strength and health-related physical fitness (HRPF). Methods Grip strength and HRPF were assessed in 569 children (12.4 years, 95% CI 12.16 to 12.72; 238 girls) with various CHD and compared with 2551 healthy controls (11.4 years, 95% CI 11.3 to 11.5; 1424 girls). Grip strength was determined as the maximum value of three repetitions with each hand. HRPF was tested by five motor tasks (FITNESSGRAM) and converted into an SD score (z-score). Results After adjusting for age, sex and weight, children with CHD showed significantly lower grip strength compared with healthy controls (CHD: 20.8 kg, 95% CI 20.4 to 21.2; controls: 24.5 kg, 95% CI 24.3 to 24.8). CHD subgroup analysis also revealed significantly lower grip strength than the controls, with the lowest values in patients with total cavopulmonary connection (19.1, 95% CI 18.0 to 20.2). Children with complex CHD showed the lowest values with 19.8 kg (95% CI 19.2 to 20.4), those with moderate 20.7 kg (95% CI 19.9 to 21.4) and those with simple 22.5 kg (95% CI 21.6 to 23.3), respectively. HRPF was also lower (z-score: -0.46, 95% CI -0.49 to -0.35) compared with healthy controls and poorly associated with grip strength (r=0.21). Conclusions Grip strength is already reduced in children with CHD and poorly associated with HRPF. This suggests that grip strength and HRPF are different domains and have to be assessed separately. Physical fitness is an important aspect of wellness. In 569 long term survivors of CHD surgery both grip strength & health-related physical fitness (a 5 parameter composite) were reduced but the reductions were unrelated. Grip strength is easily assessed but is not a surrogate for the more complex tool.