Between June 1983 and December 2002, 32 patients were treated for primary localized laryngo-tracheobronchial amyloidosis (LTBA) at our institution. For enrollment in this retrospective study, at least one positive biopsy with Congo red stain and a diagnostic test battery excluding systemic or secondary amyloidosis were mandatory. The most common presenting symptom was dyspnea, which was observed in 75% of cases. Endoscopic appearance was described as submucosal plaques and nodules with a cobblestone appearance in 44% of patients, tumor-like in 28% and circumferential wall thickening in 28% of cases. Tracheobronchopatia osteochondroplastica was associated with LTBA in 22% of patients, both at the first treatment (four cases) or during follow-up examinations (three). Synchronous or metachronous disease in the larynx and tracheobronchial tree was observed in 47% of cases, while only one patient showed lung involvement with extensive amyloidosis of the trachea and bronchi. All but two patients were endoscopically treated. One of these was affected by a tracheal amyloidoma cured by endoscopic debulking and further tracheal resection-anastomosis. The other patient presented massive laryngo-pharyngeal involvement and was therefore treated by total laryngectomy. A total of 58 endoscopic procedures (range: 1-7; mean: two per patient) were carried out, 11 with CO2 laser (for supraglottic, glottic and selected subglottic lesions) and 47 with Nd:YAG laser (for the most part of subglottic lesions and amyloid deposits located in trachea and bronchi). Four patients required a tracheotomy and three necessitated a T-tube stent during management, which was subsequently removed in all cases. One patient was lost to follow-up, nine were endoscopically controlled elsewhere (Group A) and 22 were followed at our institution (Group B). In Group A, the patient with tracheal amyloidoma is asymptomatic and free of additional LTBA localizations 8 years after surgery. The remaining eight are asymptomatic, but with persistent endoscopic signs of amyloidosis. In Group B, five patients died: one from heart failure and another one from pneumonia 3 and 4 days after surgery, respectively. In one case death was from unrelated causes, and in the two remaining patients it was from respiratory failure due to uncontrolled bilateral bronchial amyloidosis in the subsequent 5 to 6 years. One patient is completely cured 5 years after surgery, and 16 are asymptomatic with persistence of LTBA.