Primary localized amyloidosis of the nasopharynx is a rare disease. We present the case of a 13-year-old girl, to our knowledge only the second pediatric case reported in the literature. Symptoms were nasal obstruction and bleeding from the oral cavity. Physical examination revealed a mass in the nasopharynx and left side of the soft palate. The diagnosis was made by biopsy and histopathologic study of the surgical specimen. Immunohistochemical study revealed amyloid light chains (AL). The main treatment was surgical. At follow-up 9 months later, no recurrence had developed. While rare, localized amyloidosis should be considered in the differential diagnosis of nasal obstruction, epistaxis and glue ear, and must be recognized and understood by the otolaryngologist to allow appropriate diagnostic and therapeutic planning.