Respiratory tract amyloidosis is characterized by deposition of amyloid fibril protein at any site along the respiratory tract ranging from the larynx to the pulmonary parenchyma. Usually, it is not associated with primary systemic amyloidosis. We report a case with isolated laryngotracheal amyloidosis, which is relatively a rare condition, together with a history of refractory asthma accompanied with longstanding hoarseness.