It is reported about a case of congenital afirinogenaemia on a newborn
female. The treatment is described. Congenital afirinogenaemia is a r
are autosomal recessiv disorder. There are 250 cases in world literatu
re, mostly with consanguineous parents. Bleeding is usually mild, so t
hat treatment should not be prophylactic, because any blood product mi
ght cause complication, like AIDS. Only acute haemorrhage should be tr
eated with fresh frozen plasma or fibrinogen concentrates.