Rationale: Pulmonary amyloidosis is a rare respiratory disease characterized by amyloid deposition in the lungs. The clinical manifestations of pulmonary amyloidosis are variable and without specific symptoms.

Patient concerns: We report a rare case of tracheobronchial amyloidosis to improve our understanding of the disease.

Diagnoses: The diagnosis of tracheobronchial amyloidosis was finally established by transbronchoscopic lung biopsy and histological examination.

Interventions: The patient significantly improved with methylprednisolone sodium succinate for injection (40 mg/day) for 5 days and low-dose oral prednisone for 10 days.

Outcomes: After treatment, discomfort, such as cough, stridor, dyspnea, and chest tightness, disappeared, and he was discharged. The patient was in good clinical condition after 8 months of follow-up.

Conclusion: This case clearly shows that it is difficult to distinguish tracheobronchial amyloidosis from other diseases with manifestations of cough, dyspnea and chest tightness because of their similar symptoms and imaging findings. Thus, the role of transbronchoscopic lung biopsy and histological examination in the diagnosis of tracheobronchial amyloidosis is very important.