NUT carcinoma is a rare, highly aggressive cancer that feature as the rearrangement of the nuclear protein in the testis (NUT) gene on chromosome 15q14, and its pathogenesis and treatment is not yet clear. In this case, we report a 40-year-old male patient who was diagnosed with primary pulmonary NUT carcinoma in The Second Affiliated Hospital of Zhengzhou University. A tumor was found at the right hilus pulmonis when his physical examination with chest pain for half a month. Histopathology confirmed by fluorescence in situ hybridization technique for the NUT carcinoma. After chemotherapy, radiotherapy, immunotherapy, and targeted therapy were given, the patient died. The overall survival time was 4.7 months. Combined with the existing literature, we retrospective report the clinical and pathological characteristics and treatment strategies of the rare lung NUT carcinoma.