Factor VII (proconvertin) is a pivotal initial step in the activation of the extrinsic pathway of blood coagulation. Factor VII deficiency is a rare coagulopathy that can manifest in a wide range of clinical symptoms, from asymptomatic deficiency to severe hemorrhagic syndrome, which can often lead to death due to hemorrhages, including brain hemorrhages. The article provides a brief overview of the epidemiology of the disease, its clinical manifestations, diagnostic approaches, and treatment options. The authors present a case of relatively late diagnosis of hypoproconvertinemia, which manifested with severe bleeding in the neonatal period. In the first year of life, the child experienced recurrent hemorrhagic syndrome, which was managed by administering menadione sodium bisulfite trihydrate solution. Examination did not reveal deficiency of factor VIII or IX. The diagnosis was established at the age of 3 years, and the child received recombinant factor VII. However, on- demand therapy and the lack of long-term preventive administration of the drug did not provide a sufficient hemostatic effect, as evidenced by repeated episodes of hemarthrosis and severe gastrointestinal bleeding at the age of 15 years These complications necessitated a switch to continuous administration of recombinant factor VII. KEYWORDS: hypoproconvertinemia, hemophilia, children, bleeding, recombinant FVII. FOR CITATION: Dmitriev A.V., Fedina N.V., Yudakova M.S., Dronova S.N., Slabachkov K.O., Zaplatnikov A.L. Deficiency of factor VII (hypoproconvertinemia): case report. Russian Journal of Woman and Child Health. 2024;7(2):184–188 (in Russ.). DOI: 10.32364/2618- 8430-2024-7-2-15.