Wegener's granulomatosis is a systemic vasculitis characterized by necrosis, granulomatosis and inflammation. The usual targets are extra-oral, although salivary gland involvement has been recognized, usually confined to parotid and submandibular glands. A case report is presented of sublingual gland presentation, confirmed on biopsy, which proceeded to systemic involvement. Some evidence suggests that salivary gland involvement, if recognized, may lead to an early diagnosis and the potential for earlier therapeutic intervention.