{Reference Type}: Review {Title}: Successful perinatal management of a woman with congenital factor XIII deficiency using recombinant factor XIII: A case report and literature review. {Author}: Ito Y;Tsuji S;Kasahara M;Tokoro S;Murakami T;Takayama H; {Journal}: J Obstet Gynaecol Res {Volume}: 50 {Issue}: 2 {Year}: 2024 Feb 24 {Factor}: 1.697 {DOI}: 10.1111/jog.15819 {Abstract}: Factor XIII deficiency is an extremely rare autosomal recessive genetic disorder, occurring in 1 of 3-5 million people, and is associated with perinatal complications, such as habitual abortion and prolonged bleeding. Although plasma-derived factor XIII (Fibrogamin®) carries a risk of infection and contains very low concentrated forms of factor XIII (FXIII) used for a pregnant woman with congenital coagulation factor XIII deficiency, recombinant factor XIII (rFXIII, Novo Thirteen®; Tretten®, Novo Nordisk, Bagsvaerd, Denmark), which has no risk of infection and is highly concentrated, has emerged as a novel formulation. Herein, we report the first case of a Japanese pregnant woman with congenital coagulation factor XIII deficiency successfully managed by rFXIII. She had a good perinatal course without pregnancy-related complications and transfusion through the perinatal period.