Eosinophilic angiocentric fibrosis is a rare, benign cause of submucosal thickening and fibrosis within the upper respiratory tract. It predominantly affects the nose although cases have been reported in the subglottis. We describe four cases of the disease centred around the nasal cavity, with widespread infiltration of the facial soft tissues and orbit in three of the four patients. Each underwent long term follow up. Multiple surgical resections were required with two of our patients and, to date, medical therapy has been of limited help. The disease process, with its clinical and characteristic histopathological findings, is described. We also discuss the management of the disease following a comprehensive review of, and comparison with, the few prior reported cases.