Behçet's disease (BD) is a chronic relapsing inflammatory disorder of unknown aetiology with involvement of multiple organs. The incidence of BD is highest in the countries along the ancient Silk Route. In approximately 9% of the BD patients there is involvement of the central nervous system (CNS). We present a case of a 32 year-old Lebanese woman with severe headache and papilloedema due to a cerebral venous thrombosis and secondary intracranial hypertension. During follow-up she was diagnosed with bilateral uveitis and a history of recidivating oral and genital ulcerations was disclosed. BD with CNS affection is an important diagnosis to keep in mind in case of cerebral venous thrombosis.