A patient of Wegener's granulomatosis (WG) who developed intraorbital tumor without pulmonary and renal involvement was reported. A 52-year-old male has suffered from visual disturbance and right proptosis. Administration of betamethasone resulted in a temporary relief of his symptoms. However, following the reduction of steroid dosage, severe recurrence of ocular symptom occurred and his right sight was completely lost. On admission to our hospital, there were swollen eyelids and saddle nose. The CT scan demonstrated intraorbital tumors bilaterally associated with chronic sinusitis. The repeated biopsies of nasal mucosal lesions disclosed presence of a giant cell granuloma with necrotizing vasculitis. A diagnosis of WG had been made, so intermittent high-dose intravenous cyclophosphamide therapy were initiated. The size of the bilateral intraorbital tumors reduced with continuous cyclophosphamide therapy. At the last observation during 22 months after the initiation of cytotoxic therapy, there was no evidence of clinical and radiological recurrence. Three cases of WG with intracranial tumors in our hospital showed multiple cranial nerve palsy and poor response to corticosteroid therapy. The "limited form" without renal involvement has been known as a subset of WG with a relatively good prognosis. This report suggest the existence of further limited form with intracranial granuloma as a cardinal presentation of WG. Although the extent of granulomatous lesions are limited and progression is slow, the treatment by corticosteroid alone is not sufficient and as the systems form of WG, combined therapy with cyclophosphamide appeared to be indicated.