Congenital factor VII deficiency is a rare autosomal recessive hemorrhagic disorder and surgery is normally the cause of excessive bleeding. In this report, we describe the first case with congenital factor VII deficiency admitted to our clinics for the sunnet operation (circumcision), in which recombinant activated factor VII (rFVIIa; NovoSeven) was used to manage the bleeding. The patient was an 8-year old boy with moderate factor VII deficiency (factor VII level, 4%), and rFVIIa was administered at a dose of 20 mu g/kg per dose during the circumcision operation. The same dose was repeated at 2, 4, 6, 9,12,15, 18, 21 and 24 h post operation. The circumcision operation could therefore be safely performed in patients with congenital factor VII using rFVIIa.