Reports suggest that immunotherapy may represent a promising treatment technique for SMARCA4-UT.[5,6] However, a study shows that this condition primarily has an immune-desert tumor microenvironment, which restricts with limited the effectiveness of immunotherapy.[7] In this report, we describe a male patient with thoracic SMARCA4-UT responding favorably to immunotherapy (tislelizumab) combined with chemotherapy and radiotherapy. Here, SMARCA4 is linked to neoplasm.