We describe a patient in whom bilateral swelling of submandibular glan
ds was the initial manifestation of limited Wegener's disease, The dia
gnosis was based on positive anti-neutrophil cytoplasmic antibody [C-A
NCA], dense inflammatory infiltrate predominantly of lymphocytes in su
bmandibular glands, presence of non-caseating ill-formed granulomas in
transbronchial biopsy and resolution of symptoms following immuno-sup
pressive treatment.