With regards to routine histopathological workup, we identified KRT18 in combination with UCHL1 as potential immunohistochemical markers to differentiate NEC-like IDH2 and NEC-like SMARCA4/ARID1A tumors from adenoid cystic carcinomas, olfactory neuroblastomas and squamous cell carcinomas. The gene discussed is IDH2; the disease is olfactory neuroblastoma.