Even though chemotherapy was administered, she died few months later due to multiple metastases.<h4>Clinical discussion</h4>Although diagnosis is challenging in all 11 reported cases -including our case- of adult MRTK, immunohistochemistry (i.e., negative reaction for INI-1) in conjunction with clinical and radiological findings are the main tool to reach diagnosis. Here, SMARCB1 is linked to rhabdoid tumor of the kidney.