NUT midline carcinoma (NMC) represents a rare subset of highly aggressive poorly differentiated carcinomas characterized by rearrangement of the NUT (aka NUTM1, nuclear protein in testis) gene on 15q14, most commonly fused with either the bromodomain-containing 4 (BRD4) gene on 19p13.1 or the bromodomain-containing 3 (BRD3) gene on 9q34. Originally described as a mediastinal/thymic malignancy, NMC has been reported at a variety of anatomic regions including the upper and lower aerodigestive tract. NMC typically occurs in the head and neck1 or mediastinum, although more recently, there have been reports of primary lung, and renal involvement. Given the rarity and overlapping histopathologic features with other disparate tumors, the incidence of NCs remains difficult to determine. Most of these tumors occur in adolescents and young adults, arise from the midline structures of the thorax, head, and neck, and are associated with extremely poor outcomes.