The occurrence of RMC without the presence of hemoglobinopathy is even rarer (6), and this article reports the 13th case of RMC without associated hemoglobinopathy, classified according to the 4th edition of the WHO Classification of Tumors of the Urinary System and Male Genital Organs as unclassified renal cell carcinoma (RCC) with medullary phenotype and SMARCB1(INI1) deficiency (9). The gene discussed is SMARCB1; the disease is renal cell carcinoma.