Amyloidosis of the upper aerodigestive tract is rare in the pediatric age group. We present the first reported case (to our knowledge) of multifocal primary upper airway amyloidosis in a child. An otherwise-healthy 15-year-old girl presented with hoarseness, nasal congestion, and odynophagia. Diagnostic evaluation included flexible nasopharyngoscopy, rigid nasal endoscopy, direct laryngoscopy, bronchoscopy, and biopsy. The results of Congo red staining of the specimen were characteristic of amyloid. The findings of an extensive immunologic and systemic evaluation were normal. We discuss the treatment and postoperative course of this patient, as well as the clinical and pathologic characteristics of amyloidosis, with particular reference to the otolaryngologic manifestations of amyloidosis.