Amyloidosis is an orphan disease. Amyloidosis is uncommonly associated with respiratory disorders. The present article is a review of recently published data on pulmonary amyloidosis and a case report of alveolar and septal amyloid lung injury. Clinical heterogeneity of lung lesions in systemic and limited AL amyloidosis (precursor proteins are light chains of immunoglobulins, λ dimer and κ monomer), AA amyloidosis (inflammatory acute-phase proteins), and ATTR amyloidosis (senile and inherited amyloidosis; precursor protein is transthyretin) have been discussed. One chart of the review has been devoted to the pathogenesis of diffuse parenchymatous (unilateral and bilateral) amyloidosis, nodular and tracheobronchial amyloidosis (with amyloid deposits in proximal, medium and distal airways, intrathoracic lymph node involvement (amyloid lymphadenopathy), amyloidosis of the pleura and the diaphragm, and differential diagnosis of these types of pulmonary amyloidosis. © 2018 Medical Education. All rights reserved.