In this paper, we describe a 25-year-old white man with Behcet's disease who developed superior vena cava syndrome which was followed with the diagnosis of pseudotumour cerebri based on bilateral papilledema for 6 months. Complete superior vena cava obstruction was detected by magnetic resonance imaging (MRI). Secondary reasons for papilledema were all excluded. Treatment of prednisone, pulse cyclophosphamide and heparin was started and clinical symptoms and fundoscopic changes completely disappeared in 2 weeks. In conclusion, we think that Behcet's disease should always be remembered in the differential diagnosis of unidentified neurological signs especially in regions where the disease is relatively common.