RESUMO
Congenital dysfibrinogenemia is a functional disorder of the fibrinogen that represents a rare cause of thrombophilia. To report a Tunisian case of the association dysfibrinogenemia and thrombosis. A woman with ingerited dysfibrinogenemia associated with mild tendency to bleeding experienced a deep vein thrombosis of the lower-extremity at 26 years of age and a fatal pulmonary embolism a few years later. Paradoxically coagulation function of fibrinogen was markedly altered in vitro with a significally prolonged prothrombin time, activated partial thromboplastin time and thrombin time, a functional fibrinogen level that was undetected and a severely impaired fibrin polymerization. The thromboembolic events in the patient could be related to dysfibrinogenemia since the main causes of thrombophilia were excluded. Although it is rare, this cause of thrombophilia must be misdiagnosed, systematic measuring of prothrombin time, activated partial thromboplasting time and functional fibrinogen might be helpful