Eosinophilc angiocentric fibrosis is a rare fibrosing vasculitis of unknown etiology that is progressive and potentially disfiguring. It has a predilection for the upper airways presenting most commonly as an obstructing mass lesion that is diagnosed histologically. Thus far, it has been nonfatal in the more than 40 reported cases; however, subglottic and ocular involvement has resulted in significant morbidity in several patients. Treatment has been challenging with persistent disease in most cases. The current case is a prototypical presentation with a limited nasal septal lesion providing the opportunity to discuss clinically relevant issues and increase awareness. © 2010 Humana.