BackgroundSinonasal NUT carcinoma is an extremely rare, lethal malignancy with limited literature.MethodsA case series was conduction of all patients with sinonasal NUT carcinoma at a single institution between 2010 and 2022. Survival and associated were evaluated. A systematic review of the literature was performed.ResultsIn 12 patients, followed for a median of 1.5 years, the median overall survival (OS) and disease‐specific survival (DSS) were both 14.6 months. Patients with maxillary sinus tumors were 91% more likely to survive (hazard ratio [HR]: 0.094, 95% confidence interval [CI]: 0.011–0.78, p = 0.011). Patients with higher‐stage disease stage had worse OS (stage IVb‐c vs. III‐IVa, p = 0.05). All three patients who were alive with no evidence of disease received induction chemotherapy.ConclusionFor patients with sinonasal NUT carcinoma, the median survival was 15 months but better with lower‐stage and maxillary tumors. Induction chemotherapy may be beneficial.