Recently, several authors have shown that OSCCHT is characterized by the inactivation of the SMARCA4 gene (encoding the BRG1 protein), a member of the SWI/SNF chromatin-remodeling gene complex, which is also frequent in malignant rhabdoid tumors, resulting in a loss of BRG1 protein expression with IHC.[12–15] Thus, the absence of SMARCA4/BRG1 immunostaining may prove very useful in the diagnosis of OSCCHT.[12,15] In the present case, the tumor cells were negative for BRG1 protein, which further supported the final diagnosis of OSCCHT. The gene discussed is SMARCA1; the disease is neoplasm.