Given the unique renal pathology of a high grade malignancy with loss of SMARCB1 expression via immunohistochemistry, and history of meningioma with MLH1 loss of expression and retained expression of PMS2, MSH2 and MSH6, further germline genetic testing was sent for <i>SMARCB1</i> and mismatch repair syndromes. The gene discussed is MLH1; the disease is meningioma.