Tracheobronchial amyloidosis (TBA) is a rare idiopathic disorder resulting from the deposition of fibrillar proteins in the tracheobronchial tree. Amyloid is an extracellular deposit, which characteristically appears as a homogeneous, pink, acellular material under a light microscope. These deposits are often surrounded by fibrosis, as seen in our patient’s biopsy specimens. A diagnostic apple green birefringence is seen when a specimen is stained with Congo red stain and viewed under polarized light. TBA represents one of three recognized types of localized AL pulmonary amyloidosis. These localized amyloid deposits are characterized by their location and presentation. These deposits are then categorized as nodular, diffuse, or tracheobronchial. Tracheobronchial disease is further subdivided into one of the following two described types: diffuse infiltrative; or nodular. Nodular TBA is by far the least common of the two types. A literature review in 2006 identified only 20 cases of nodular TBA worldwide. Nodular TBA, as seen in our patient, is then described as proximal, middle, or distal airway disease based on the location of amyloid deposits in the airway.