Amyloidosis of the upper aerodigestive tract is rare in the pediatric
age group. We present the first reported case (to our knowledge) of mu
ltifocal primary upper airway amyloidosis in a child. An otherwise-hea
lthy 15-year-old girl presented with hoarseness, nasal congestion, and
odynophagia. Diagnostic evaluation included flexible nasopharyngoscop
y, rigid nasal endoscopy, direct laryngoscopy, bronchoscopy, and biops
y, The results of Congo red staining of the specimen were characterist
ic of amyloid. The findings of an extensive immunologic and systemic e
valuation were normal. We discuss the treatment and postoperative cour
se of this patient, as well as the clinical and pathologic characteris
tics of amyloidosis, with particular reference to the otolaryngologic
manifestations of amyloidosis.