A 56-year-old patient with an uneventful history was hospitalized for investigation of recent dypnea. The physical examination was normal and chest X-ray showed localized interstitial lesions in the upper right lobe. Endoscopy showed evidence of diffuse infiltration of the bronchial mucosa and narrowing of the lumen. Transbronchial histology showed evidence of bronchopulmonary amyloidosis. There were no signs of extrapulmonary localizations. Search in other organs was negative demonstrating the primary nature of the lung disease. Primary bronchopulmonary amyloidosis is rarely encountered.