Immunohistochemistry revealed the complete absence of the SMARCB1/INI-1 protein, and genetic testing identified a novel germline mutation in the SMARCB1/INI-1 gene in both the patient and his father, suggesting a "second-hit loss." One year of follow-up after the tumor's radical resection revealed no suspected metastasis. Here, SMARCB1 is linked to neoplasm.