Inherited Factor V deficiency is a rare bleeding disorder. We describe the genetic analysis and anesthetic management of a parturient with severe Factor V deficiency who presented in spontaneous labor. Good hemostatic conditions were obtained with prophylactic fresh frozen plasma administration of 10 mL/kg. Detailed genetic analysis by next-generation sequencing identified several relevant mutations in the coding part of the Factor V (F5) gene in our patient, her parents, and the newborn.