Central nervous involvement (CNS) is uncommon manifestation of Wegener's granulomatosis (WG). We are describing a patient with refractory WG with CNS involvement which responded to rituximab. This lady presented with nodular scleritis. Three months later, she developed headache, vision loss with complete opthalmoplegia, and relative afferent papillary defect. The brain MRI showed thickened dura along ant temporal region and enlargement of right cavernous sinus. C ANCA and PR 3 ELISA were positive. Non-contrast CT scan of orbit and paranasal sinuses showed soft tissue swelling of the right orbit along with mucosal thickening of right maxillary sinus. There were bilateral upper lobe nodules on HRCT of the chest. She responded to 3 days of methylprednisolone pulses and 1 g pulse cyclophosphamide but had multiple relapses while receiving high dose oral steroids and pulse cyclophosphamide. Then she was given four infusions of rituximab (375 mg/m(2)) at one-weekly interval. She had complete remission following rituximab. She relapsed after 6 months but again responded to repeat rituximab infusion.