Factor XII (FXII) is an important protease that plays a major role in the i
nitiation of the intrinsic pathway of blood coagulation. Although congenita
l FXII deficiency is not associated with a clinical bleeding tendency, it c
an be identified on a routine coagulation test, such as a prolonged activat
ed partial thromboplastin time. This deficiency is a rare autosomal recessi
ve disorder. It is still unclear whether FXII deficiency causes any disorde
rs during pregnancy. Recurrent miscarriages and placental abruption were re
ported in cases with FXII deficiency. We successfully treated a woman whose
pregnancy was complicated by congenital FXII deficiency. We report her cli
nical courses of gestation, delivery, and puerperium and discuss the role o
f maternal FXII associated with pregnancy. In our case, courses of gestatio
n and delivery were normal. Postpartum uterine bleeding was, however, prolo
nged due to a subinvolution of the puerperal uterus. Our results indicate t
hat, except for postpartum uterine contraction, FXII does not play a major
role in gestation and delivery. We suggest that FXII deficiency is not asso
ciated with recurrent miscarriage and that normal gestation and vaginal del
ivery are possible even in cases with congenital FXII deficiency. We assert
that the possible correlation of FXII deficiency with recurrent miscarriag
e merits reevaluation.