Children with congenital heart disease are at risk for developing increased arterial stiffness and this may be modulated by physical activity. Objective: To compare arterial stiffness in high-and low-physically active children with congenital heart disease and healthy age-and sex-matched controls. Patients: Seventeen children with congenital heart disease (1262 years; females=9), grouped by low-and high-physical activity levels from accelerometry step count values, and 20 matched controls (1163 years; females=9) were studied. Outcome Measures: Carotid-radial pulse wave velocity was assessed with applanation tonometry to determine arterial stiffness. Body composition and 6-min walk test measures were performed. Data were analyzed using analysis of variance and multiple regression. Significance was P<.05. Results: Arterial stiffness was increased in low-physically active children with congenital heart disease (9.7960.97 m/s) compared to high-physically active children with congenital heart disease (7.8860.71 m/s; P=.002) and healthy-matched controls (8.6761.28 m/s; P=.015). There were no differences in body composition measures between groups (all P>.05), but 6-min walk test distance was less in both congenital heart disease groups (high-physically active: 514 +/- 40 m; low-physically active: 539 +/- 49 m) versus controls (605 +/- 79 m; all P<.05). Average daily step count significantly predicted arterial stiffness in children with congenital heart disease (R-2=0.358) with a negative correlation (R=-0.599, P=.011), while % fat mass (P=.519) and % lean mass (P=.290) did not predict arterial stiffness. Conclusions: Low-physically active children with congenital heart disease have increased arterial stiffness compared to high-physically active children with congenital heart disease and healthy-matched controls. Regular physical activity in children with congenital heart disease may modulate arterial stiffness.