Seven cases of localized amyloidosis limited to structures of the head and neck and upper aerodigestive and lower respiratory tracts evaluated and treated at Boston University Hospitals in a recent 7-year period were reviewed. Negative Congo red staining of abdominal adipose aspiration biopsy or rectal biopsy specimens established that the amyloidosis was not systemic. Localized amyloidosis occurred in discrete masses in a variety of sites in the aerodigestive tract including the orbit, nasopharynx, lips, floor of mouth, tongue, larynx, and tracheobronchial tree. Five patients required surgical excision because of significant airway obstruction or organic dysfunction. Amyloid deposits completely excised with the carbon dioxide laser have not recurred, though other amyloid masses may appear elsewhere within the same organ or region. Amyloidosis may occur primarily or secondarily to other disease states. Localized amyloidosis has not been chemically identified but is usually defined by the absence of systemic features. While rare, amyloidosis must be recognized and understood by the otolaryngologist/head and neck surgeon to allow appropriate diagnostic and therapeutic planning.