High-grade renal cell carcinoma with morphology and immunophenotypic features identical to renal medullary carcinoma, occurring in a patient without evidence of sickle cell trait or disease, is proposed to be diagnosed as renal cell carcinoma unclassified with medullary phenotype (RCCU-MP) and classified as a subtype of SMARCB1-deficient renal medullary carcinoma in the World Health Organization (WHO) 2022 edition. So far, about twelve tumors of RCCU-MP have been reported in the literature. Herein, we extend this observation to a tumor of RCCU-MP in a 39-year-old patient, morphologically similar to renal medullary carcinoma. SMARCB1 expression was absent by immunohistochemistry, but there was no evidence of hemoglobinopathy. The tumor cells were positive for keratin19, PAX8 and PAX2. GATA3, OCT3/4, and ALK were negative. Fluorescence in situ hybridization was conducted to detect the