NUT midline carcinoma is a rare, highly aggressive tumor that involves midline structures, particularly in the head, neck and mediastinum. It is characterized by NUT gene translocations on chromosome 15. It typically impacts teenagers or young adults, and has a fulminant course leading to death in less than a year in most cases despite aggressive chemoradiotherapy. Due to its location, this tumor is frequently considered inoperable. We present a case of a sinonasal NUT midline carcinoma with orbital invasion discovered during the workup of sinusitis in a young, pregnant woman. The tumor was managed with definitive excision to negative margins followed by aggressive chemoradiation, with no evidence of recurrence for 12 months. We propose that diagnosis of NUT midline carcinoma should prompt recognition of the limitations of current medical therapy and rapid surgical intervention should be undertaken when possible
