Abstract: Congenital dysfibrinogenemia (CD) is a group of disorders characterized by structural and functional abnormalities of fibrinogen resulting from mutations in the FGA, FGB, or FGG genes. Its clinical phenotype is highly heterogeneous, ranging from asymptomatic presentations to bleeding or thrombosis, with a minority of patients exhibiting both bleeding and thrombotic manifestations concurrently. This review focuses on thrombosis-associated mutations in CD, summarizing their clinical features, laboratory and molecular diagnostic approaches, functional research evidence, and principal prothrombotic mechanisms. On this basis, the advances in the clinical management of CD are outlined, with the aim of providing a reference for thrombotic risk assessment and individualized management of patients with CD.