Salivary gland involvement is a rare clinical feature of Wegener's granulomatosis. We report a series of five cases in which submandibular or parotid gland swelling was part of the initial manifestation of the disease. All patients had limited forms of Wegener's granulomatosis. The value of determination of serum anti-neutrophil cytoplasmic autoantibodies is demonstrated in four of the cases, and its use as a new diagnostic adjunct and promising tool to monitor disease activity is discussed. The use of trimethoprim-sulfamethoxazole as part of alternative treatment regimens for limited Wegener's granulomatosis is advocated.