Localized amyloidosis is characterized by the deposition of amyloid fibres in a particular site or organ system in the absence of systemic involvement. Patients with localized laryngeal amyloidosis usually present with long-standing hoarseness or dyspnea. The diagnosis is made by a high degree of suspicion on the basis of the history and a characteristic appearance on direct laryngoscopic examination. When such lesions are seen, an adequate deep punch biopsy should be obtained, and an experienced pathologist should be able to identify the lesion on routine staining. However, the slides should be stained with Congo red and examined with polarized light microscopy to establish the diagnosis. Following proper diagnosis and evaluation of the extent of disease, usually by computed tomographic scan, surgery is the treatment of choice. Preservation of the voice and airway should be the aim in all patients. Endoscopic carbon-dioxide laser excision of the mass should be the first line of therapy. Patients may require repeated removal of the amyloid deposits. The results of treatment are excellent.