We present what we believe is the first reported case of a patient with supraglottic stenosis secondary to Wegener granulomatosis. The diagnosis was unclear initially because the biopsy results were nonspecific, but a finding of an elevated cytoplasmic-pattern antineutrophil cytoplasmic antibody (c-ANCA) level established the diagnosis of localized supraglottic Wegener granulomatosis. Wegener granulomatosis is characterized by necrotizing vasculitis that is localized predominantly to the kidneys and the upper and lower airways. In the airways, subglottic involvement is well documented, but to the best of our knowledge, supraglottic stenosis has not previously been described. Localized forms of Wegener granulomatosis are characterized by limited disease that involves only the upper airway. The diagnosis in localized forms is complex because histology is diagnostic in only 50% of cases, and only 60% of patients have a positive c-ANCA level. We discuss the diagnostic criteria and management strategies for these localized forms.