An association between dural arteriovenous fistula and cerebral sinus thrombosis is reported. It is clear in several cases that thrombosis precedes the development of the fistula while it is unclear that it occurs in every case. The authors report a case of a woman with sinus thrombosis and presence of prothrombin gene mutation who subsequently developed a large dural arteriovenous fistula. Various possible factors involved in the pathogenesis of a dural fistula are discussed, with emphasis on underlying thrombophilia and oral contraceptive use in this patient.