The differential diagnosis is broad and should include adenoid cystic carcinoma, basaloid squamous cell carcinoma, adenosquamous carcinoma, high-grade myoepithelial carcinoma, biphenotypic sinonasal sarcoma, NUT carcinoma, SMARCB1-deficient sinonasal carcinoma, and renal cell-like adenocarcinoma (2, 10, 13). The gene discussed is SMARCB1; the disease is nut midline carcinoma.