Salivary gland involvement is rare in Wegener's granulomatosis. We report the first case of widespread major salivary gland enlargement as part of the presentation of this disease. A review of the few reported cases in the literature suggests that salivary gland involvement may be associated with a limited form of the disease and an improved prognosis. The anti-neutrophil cytoplasmic antibody (c-ANCA) assay remains the gold standard of diagnosis but care should be exercised in the interpretation of results. This patient responded well to current immunosuppressive therapy.