We describe a patient with neuro-Behçets disease (NBD) that presented with symptoms of raised intracranial pressure including papilloedema. MRI revealed tumour-like lesions which, on biopsy, confirmed an active vasculitis. Treatment was commenced with prednisone and cyclophosphamide which proved unsuccessful with enlargement of the cerebral mass lesions. Infliximab and mycophenolate were trialled also without benefit. The patient required ventriculoperitoneal shunts to relieve the symptoms of hydrocephalus. Rituximab was then commenced with significant symptomatic and imaging improvement. The case is unique, in our experience, in the need for shunting to relieve the symptoms of hydrocephalus related to vasculitis.