Amyloidosis confined to the trachea is an exceedingly rare entity. We describe the case of a 63-year-old man who presented with a history of dysphonia and stridor. Rigid bronchoscopy revealed a segment of abnormal tissue at the midtracheal level, resembling granulation tissue. A stent was placed in an attempt to secure the patient's airway, which was >50% narrowed. Although the patient's stridor disappeared completely, 5 days postoperatively it recurred, worsening within hours. Emergency bronchoscopy revealed that the tracheal stent was almost completely obstructed with amyloid and granulation tissue, despite high-dose steroid therapy, and had to be removed. Tracheostomy was performed to bypass the diseased trachea. We also highlight some of the problems encountered with tracheal stenting in benign tracheal disease.