The clinical and pathological characteristics, possibility of systemic disease, and effect of local therapy were studied in laryngeal amyloidosis. Records of all patients with localized laryngeal amyloidosis in a single tertiary referral center were examined retrospectively at diagnosis and after local therapy. Of 188 new patients with amyloidosis between 1990 and 2003, 5 patients had localized laryngeal amyloidosis. A sixth patient with localized laryngeal amyloidosis turned out to have systemic AL (immunocyte-derived) amyloidosis 8 years later. Free light chains were found in this patient, as well as in 1 of the other 5 patients. Amyloid interfering with laryngeal or airway function was removed during microlaryngoscopy with a carbon dioxide laser or cold endoscopic excision. The best results were seen when glottic deposits were removed by cold endoscopic excision, and supraglottic deposits by a carbon dioxide laser. Four patients had recurrent disease. A systematic workup, including measurement of free light chains, helps to rule out systemic disease.