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Inherited FVII deficiency arise from mutations in the <jats:italic>F7<\/jats:italic> gene, resulting in a highly heterogeneous and wide range of phenotypic expressions from asymptomatic individuals to those experiencing severe and life\u2010threatening hemorrhagic episodes. Notably, the severity of symptoms is typically not correlated with plasma FVII levels. Diagnosis generally involves a combination of coagulation assays, including prothrombin time (PT), assessment of FVII activity and antigenic levels, and a thorough consideration of the patient's clinical context. Management of FVII deficiency requires personalized treatment strategies. For inherited forms, on\u2010demand replacement therapy is commonly employed, utilizing recombinant activated FVII, plasma\u2010derived FVII, prothrombin complex concentrates, or fresh frozen plasma during bleeding episodes, as well as for prophylactic measures. Long\u2010term prophylaxis is particularly critical for patients with severe deficiencies who have a history of central nervous system or gastrointestinal bleeding, especially those at an elevated risk of subsequent major or potentially life\u2010threatening bleeding events. This review evaluates current therapeutic approaches, highlights emerging challenges in the management of FVII deficiency, and underscores the importance of tailored treatment strategies aimed at optimizing patient outcomes.<\/jats:p>","DOI":"10.1002\/pbc.31888","type":"journal-article","created":{"date-parts":[[2025,6,26]],"date-time":"2025-06-26T22:54:19Z","timestamp":1750978459000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Coagulation Factor VII Deficiency: A Review of the Clinical Implications and Approaches to Replacement Therapy and Prophylaxis"],"prefix":"10.1002","volume":"72","author":[{"given":"Nader","family":"Cohan","sequence":"first","affiliation":[{"name":"Hematology Research Center Shiraz University of Medical Sciences  Shiraz Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elham","family":"Abedi","sequence":"additional","affiliation":[{"name":"Hematology Research Center 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