Fumarate hydratasedeficient renal cell carcinoma(FH-RCC)is s a rare and highly aggressive and metastatic form of renal cell carcinoma. Clinical diagnosis is relatively challenging, and there is a lack of recognized systemic treatment options. Therefore, in clinical practice, it is necessary to have a deeper understanding of this disease and fully integrate family history, imaging findings, and pathological manifestations for clinical identification, and genetic testing shoule be conducted when necessary. Improved patient prognosis can be achieved through the selection of appropriate curative surgery or systemic treatment strategies.