Midline carcinoma with nuclear protein in testis(NUT)gene rearrangement,also known as NUT carcinoma,is a rare and highly aggressive malignant tumor characterized by young onset,rapid progression,and poor response to conventional treatments,leading to a generally poor prognosis for the majority of patients.The pathogenesis of NUT carcinoma is associated with rearrangements of the NUT midline carcinoma family member 1(NUTM1)gene,where the resulting fusion genes block cellular differentiation and promote cancer cell growth.Insufficient clinical understanding of this disease currently results in many patients being misdiagnosed or undiagnosed.This article summarizes previous reports on the clinical pathological characteristics,pathogenesis,and relevant diagnostic and treatment methods of NUT carcinoma to enhance clinical awareness of the disease.For patients with initial diagnoses of poorly differentiated carcinoma accompanied by rapid tumor progression or distant metastasis,it is recommended to actively improve NUT immunohistochemical examinations.For those highly suspicious cases,genetic sequencing should be performed to determine the fusion partner type.Given the inadequate efficacy of conventional treatments,the best treatment approach involves formulating treatment plans through multidisciplinary consultations and adjusting them promptly according to changes in the patient's condition.