To establish the pharmacokinetic profile of activated recombinant coagulation factor VII (rFVIIa; NovoSeven®) in children with haemophilia A, and to compare it with the pharmacokinetic profile in adults with haemophilia A. Twelve children (2–12 years) received one single dose of rFVIIa 90 and 180 μg kg−1 in randomized order separated by a washout period of 48 h to 1 month. Six adults (18–55 years) received a single dose of rFVIIa 90 μg kg−1. The pharmacokinetic analyses were based on a non-compartmental method. In children, the plasma level of FVII increased proportionally with the dose. The total body clearance normalized for body weight was significantly faster in children than in adults (FVII:C, 58 vs. 39 mL kg−1 h−1 and FVIIa, 78 vs. 53 mL kg−1 h−1, P < 0.05). A trend towards a larger volume of distribution at steady-state in children than in adults was observed (P > 0.05). Dose proportionality was established for plasma concentrations of FVII in children with haemophilia A at the dose levels investigated (90 and 180 μg kg−1 rFVIIa). Following administration of rFVIIa 90 μg kg−1, significantly faster clearance was observed in children compared with adults, suggesting that higher doses of rFVIIa may be needed to achieve the same plasma levels as in adults.