Amyloid is defined as a pathologic proteinaceous substance which, when depo
sited between the cells of tissues and organs, leads to various clinical co
nditions. Immunohistochemistry has allowed for better classification and un
derstanding of the pathophysiology of amyloidosis. In the upper aerodigesti
ve tract, amyloidosis is a rare condition occurring most frequently in the
larynx. We present the case of a 42-year-old woman with complete nasal obst
ruction due to primary nasopharyngeal amyloidosis. This represents the firs
t reported case of primary nasopharyngeal amyloidosis containing both the l
ambda and kappa immunoglobulin light chains. The clinical and radiologic fi
ndings, as well as the management of primary amyloidosis of the upper aerod
igestive tract, will be discussed. A review of the literature pertaining to
nasal and nasopharyngeal amyloidosis will be presented.