Congenital afibrinogenemia is a rare inherited autosomal recessive disorder in which a mutation in one of three genes coding for the fibrinogen polypeptide chains Aα, Bβ or γ results in the absence of a functional coagulation protein. A patient with congenital afibrinogenemia, due to a FGA homozygous gene deletion, underwent an orthotopic liver transplantation that resulted in complete restoration of normal hemostasis. The patient's explanted liver provided a unique opportunity to further investigate a potential novel treatment modality.