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Localized tracheobronchial amyloidosis is a rare disease that results from submucosal deposition of insoluble amyloid proteins in the large airways. Amyloidosis affecting the larynx and subglottic space typically results in unilateral, nodular vocal cord infiltration. It rarely can present with bilateral vocal cord involvement and can progress to lifethreatening respiratory failure due to upper airway obstruction. In these patients, typical treatment modalities such as CO2 laser ablation are often ineffectual. Bronchiectasis is a predisposing risk factor associated with the secondary (AA) form of disease. We present a patient with the primary (AL) form of localized laryngotracheal amyloidosis with pre-existing bronchiectasis, and also review the existing literature on this disease.