Wegener's granulomatosis is difficult to diagnose, especially when the presentation is unusual, restricted to an isolated region. We report four cases of recurrent subglottic stenosis posing difficulty in diagnosis. In each case the finding of anti-neutrophil cytoplasm antibodies (ANCA) strongly suggested an underlying vasculitic pathology, Wegener's granulomatosis. We discuss reasons for the difficulty in diagnosis in the past, the possible role of the ANCA assay in such patients, and suggest it should be more widely used in the future.