BACKGROUND: Factor VII deficiency is an autosomal recessive bleeding disorder with an incidence of one in 500,000. Few cases have been reported in pregnancy, and only two patients were treated with recombinant factor VIIa. In the past, fresh frozen plasma or factor VII concentrate has been the conventional treatment for this disorder. CASE: We report a case of factor VII deficiency diagnosed during pregnancy with a factor VII level of 1%. After prophylactic treatment with recombinant factor VIIa, the patient did not manifest any signs or symptoms of excessive bleeding during labor or postpartum. CONCLUSION: Given the inherent risks of transmission of human immunodeficiency virus and hepatitis with blood products, recombinant factor VBa treatment is an alternative that is safe and effective during labor, delivery, and the puerperium.