We present, to our knowledge, the first reported case of germline neurofibromatosis Type 2 (NF2) associated with renal cell carcinoma unclassified with medullary phenotype (RCCU-MP) with somatic loss by immunohistochemistry of the <i>SMARCB1</i> tumor suppressor gene located centromeric to NF2 on chromosome 22q. The gene discussed is SMARCB1; the disease is renal cell carcinoma.