Vasculitis with central diabetes insipidus is a rare condition which must be recognized for proper management. We report a case of Wegener's granulomatosis with diabetes insipidus and review the literature concerning the clinical and radiological features of this association. We examined the data supporting the efficacy of cyclophosphamide in diabetes insipidus. Diabetes insipidus is inaugural in 46% of cases. Wegener's granulomatosis has no distinctive features when associated with diabetes insipidus. MR imaging reveals an enlargement of the posterior pituitary in 100% of cases. Cyclophosphamide is effective in 80% of cases. In Wegener's granulomatosis, pituitary vasculitis is a rare condition. MR imaging is the most accurate method for pituitary exploration. Cyclophosphamide is effective in the treatment of vasculitis lesions in Wegener's granulomatosis.