An inflammatory myofibroblastic tumor (IMT) is an immunohistochemically diverse entity demonstrating neoplastic and nonneoplastic qualities. Although IMTs can arise in any area of the body, lesions arising in certain sites, namely, the nasal cavity, paranasal sinuses, and pterygopalatine fossa, demonstrate a heightened neoplastic and invasive potential. Despite case specific complete tumor regression and disease remission in response to pharmacotherapeutics, a subset of IMTs remain resistant to all forms of therapy. We present such a case, a 34-year-old female patient, with a highly resistant, maxillary sinus IMT. Her refractory, ALK-1 negative IMT has not responded well to novel therapies reported in current literature. This case suggests the role of zonal expressivity within a single lesion as a probable mechanism for its highly resistant nature and should promote determination of each IMT's cytogenetic profile to provide more effective targeted therapy. Paper includes a literature review of all maxillary sinus IMTs from 1985 to 2014 along with their immunohistochemical staining, treatments, and outcomes.