NUT carcinoma (NC) is an exceedingly rare and poorly differentiated carcinoma characterised by BDR4:NUTM1 gene translocation. It typically affects young adults, and due to its dismal clinical course, standardized therapeutic recommendations are lacking. In this study, we present a successful multimodal treatment approach for a 13-year-old boy diagnosed with primary chest NC harboring a novel NUTM1 rearrangement, achieving complete continuous remission 21 months after diagnosis.