Wegener's granulomatosis is a systemic necrotizing vasculitis affecting small to medium-sized vessels, characterized by involvement of the upper airways, lungs and kidneys. Endocrine involvement, particularly the thyroid gland is extremely rare. We report a 56-year-old woman presenting a limited form of Wegener's granulomatosis with the following clinical manifestations: sinusitis, pulmonary nodules, diabetes insipidus and a cold thyroid nodule. A right hemithyroidectomy was performed and the histopathological examination revealed a necrotizing vasculitis surrounded by granulomatous lesions. She was treated by corticosteroids without immunosuppressive therapy with a favourable outcome.