The clinicopathological features and immunophenotype of 3 cases of succinate dehydrogenase deficient renal cell carcinoma (succinate dehydrogenase-deficient renal cell carcinoma, SDH-deficient RCC) were analyzed by HE staining and immunohistochemistry (IHC), and combined with relevant literatures. SDH-deficient renal cell carcinoma occurred both in men and women in 3 cases, with an average age of 39.33 years old, ranging from 22 to 48 years old. The lesion was located in the left kidney in 1 case and in the right kidney in 2 cases. One patient had two renal masses on the same side, located at the upper pole of the right kidney and the lower pole of the right kidney respectively. All tumors are generally well circumscribed, without definite capsule, with pushing growth at the margin, solid section, homogeneous, grayish red or grayish brown. Microscopically, the tumor tissue structure is diverse, with nests, small tubes, acini or solid structures. The tumor cells are polygonal or round, with unclear cell boundaries, abundant cytoplasm, eosinophilic or transparent, vacuolar or inclusion body structures in the cytoplasm. Eosinophilic flocs, round or oval nuclei, smooth and regular nuclear membrane, fine chromatin, inconspicuous nucleoli, and relatively consistent nuclear size as low-grade nuclei are also its presented characteristics. Normal renal tubules or glomeruli can be seen around the tumor. Immunohistochemistry showed that the expression of SDH subunit B (SDHB) antibody was absent, and the surroundings of the mass were non-tumor tissues. Moreover, renal tubules, vascular endothelial cells and inflammatory cells were all positive in expression. Three patients were followed up for 2 to 8 months after surgical treatment and all survived. SDH-deficient renal cell carcinoma has typical histopathological cell characteristics, and its diagnosis needs to be confirmed by immunohistochemical markers.