Abstract: Objective To improve the understanding of Wegener’s granuloma（WG） and WG-involvd pituitary. Methods A case of diabetes insipidus induced by WG-involved pituitary admitted to our hospital in 2009 was reported.Its clinical manifestations,diagnosis and treatment were discussed in view of its relevant foreign and domestic literature. Results The patient was a 59 years old female.Her initial clinical manifestions were polydipsia and polyuria which were relieved after treatment with desmopressin,followed by fever,headache,rhinocleisis,nasal discharge,earache,hearing impairment,ophthalmalgia and blurred vision which were significantly impoved after treatment with corticosteroid and cyclophosphamide.Laboratory test showed positive serum antineutrophil cytoplasmic antibodies（ANCA）,CT scan displayed solid tissues in maxillary sinus and a small nodule in the superior lobe of left lung,MRI revealed pituitary gland enlargement and infundibulum thickening with significant enhancement,and histopathology demonstrated inflammation and granuloma in paranasal sinuses.More than 20 cases of WG-involved pituitary were reported in the literature,most of them manifested as central diabetes insipidus and hypopituitarism. Conclusion WG is an ANCA-related multi-system necrotizing granulomatous vasculitis usually involving the upper and lower respiratory tract and kidneys and occasionally involving pituitary,which induces central diabetes insipidus and hypopituitarism.