NUT midline carcinoma, characterized by the rearrangement of the nuclear protein in testis (NUTM1) gene, is a rare and aggressive subtype of squamous cell carcinoma. This disease is rarely cured and there have been no reports of cure in patients with distant metastatic disease. In fact, patients typically succumb to NUT midline carcinoma within 6 to 12 months from diagnosis. The authors report on a single patient who presented widely metastatic disease who has now been in remission for 37 months after multimodal therapy with compressed cycles of vincristine, cyclophosphamide, and doxorubicin alternating with ifosfamide and etoposide, high-dose radiation, and postchemotherapy resection.