Wegener's granulomatosis is a granulomatous necrotizing vasculitis which predominantly affects the respiratory tract, kidney, and less frequently other organs such as the nervous system. The latter may occur in up to 54% of cases and when it does it is more frequently of the peripheral nerves. We present a 19 year old woman who commenced her disease with involvement of respiratory sinuses, lungs and kidney and who developed central insipid diabetes (CID) at onset. The CID persisted in spite of adequate response of the other organs and systems with immunosuppresor treatment. The development of CID in the context of vasculitis should suggest this as a possible mechanism.