Primary laryngo-tracheobronchial amyloidosis is a rare pulmonary disease for which there is currently no established treatment. Herein, we report a case involving a 51-year-old woman who was admitted to hospital primarily due to intermittent hoarseness. Botryoidal masses were visible at the entrance of the throat. On chest computed tomography, eccentric soft tissue density shadows were apparent in the tracheal bifurcation of the proximal segment of the trachea, and the lumen was narrowed. Electronic bronchoscopy revealed extensive new biological invasive growth from the nasopharynx to the glottis. The glottis and larynx were irregularly shaped with clear characteristics and congestion. New biological growth was also observed in the right upper lobe, right middle bronchus, and right lower lobe mucosa. The patient was ultimately diagnosed with primary laryngo-tracheobronchial amyloidosis via pathological analysis. Although amyloidosis is a benign lesion, to date, there are no curative treatments. The present article briefly highlights the pertinent literature, and discusses the clinical manifestations and progress of treatment in primary laryngo-tracheobronchial amyloidosis.