Objective: We report an extremely rare case of isolated primary sinonasal amyloidosis.Method: A case report and comprehensive literature review are presented. Common symptoms, signs and treatment options are examined, and used to derive an algorithm with which the practising otolaryngologist can diagnose and manage this condition.Results: Only 15 cases of true primary idiopathic amyloid restricted to the sinonasal tract have previously been reported. Isolated sinonasal amyloid presents with common rhinological symptoms which mimic chronic rhinosinusitis, i.e. nasal obstruction, nasal discharge, epistaxis, 'glue ear' and post-nasal drip. The lesion itself can appear as a simple inflammatory polyp. In children, the symptoms are easily attributed to enlarged adenoids. Although there is a concern that surgically induced trauma to the paranasal mucosa might incite reactive amyloid deposition, this seems not to be the case, and surgery provides symptomatic relief in the majority of patients.Conclusion: This article is the first to report the characteristic features of isolated primary sinonasal amyloidosis. The evidence suggests that meticulous surgery can produce substantial symptomatic improvement in these patients.