SMARCB1-deficient sinonasal carcinoma was first described and reported in 2014, and was first recognized as a subset of sinonasal undifferentiated carcinoma in the 4th edition of the World Health Organization’s Classification of Head and Neck Tumors in 2017 [1,2,4]. This evidence concerns the gene SMARCB1 and nasal cavity and paranasal sinus carcinoma.