NUT carcinoma is a rare, aggressive malignancy characterized by chromosomal rearrangements involving the NUTM1 gene. We present the case of a 47-year-old male with a history of hypertension, diagnosed with NUT carcinoma originating in the ethmoid sinus and accompanied by extensive neck metastases. The patient underwent a left modified radical neck dissection and endoscopic nasal tumor excision, followed by concurrent chemoradiotherapy. Pathological examination confirmed NUT carcinoma with metastatic cancer cells in 41 out of 72 lymph nodes of left neck, along with extranodal extension. The patient remained alive at the 4-month follow-up. This case highlights the importance of early identification and including NUT carcinoma in the differential diagnosis to optimize treatment outcomes.
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