Aim: Nasopharyngeal amyloidosis is rare, with very few reports in the literature. We report a case of amyloidosis of the nasopharynx extending into the larynx mimicking a tumoral process and compromising the airway, a unique clinical presentation. Background: Amyloidosis is a condition characterized by the deposition of extracellular proteinaceous material, resulting in damage to the tissues. A total of 19% of cases involve the head and neck with the oropharynx and larynx being the most affected organs. Localized amyloidosis is a rare and slow-growing lesion and is considered benign tumor, but it may be aggressive locally. Case description: A 49-year-old female presented with voice change and breathlessness. On clinical evaluation, multiple swellings involving the nasopharynx and larynx compromising the airway were noticed and a biopsy was done, which confirmed our primary clinical suspicion of Amyloidosis and was planned for staged coblation-assisted excision of the lesions to secure adequate airway. Conclusion: A complete clinical and paraclinical work-up is required for the positive diagnosis of primary or secondary amyloidosis. Morbidity is high so postsurgical follow-up is essential. Clinical significance: The localized form usually follows a slow and benign clinical course with a favourable prognosis of 90.6%. The principal differential diagnosis is a malignant tumor, hence the importance of histopathologic diagnosis. The recurrence rate approximates zero, but the extremely rare presentation and slow progression of this pathology make surveillance mandatory.