A 43-year-old man suffered generalized convulsions following severe headache. Initial angiography showed a dural arteriovenous fistula (DAVF) involving the right transverse-sigmoid sinus, and no contrast filling of the superior sagittal sinus, bilateral transverse sinuses, the left sigmoid sinus and the straight sinus. The diagnosis was extensive sinus thrombosis associated with DAVF. Transarterial embolization was performed for the DAVF. The follow-up angiography at 7 days postembolization showed no contrast filling of the DAVF. But the follow-up angiography at 6 months disclosed the appearance of multiple DAVF's involving the superior sagittal sinus, the left transverse sinus and the straight sinus. Each DAVF was supplied by different dural arteries. Coagulation tests revealed deficiency of plasma protein S. The pathogenesis of DAVF is controversial. But our case suggests that multiple isolated lesions indicated the presence of a congenital dural vascular abnormality, which was transformed to multiple DAVF's by sinus thrombosis.