Abstract: Objective: To analyze the clinico-pathological characteristics, pathological diagnosis, and treatment of rhabdoid tu -mor. Methods:The medical records of four rhabdoid tumor patients that were admitted to the Tianjin Medical University Cancer Insti-tute and Hospital since 2000were analyzed based on existing literature.Results:In one of the four cases, the tumor originated from the kidney, whereas in the other three, the tumor occurred from extra-renal soft tissues. Histologic analysis revealed that the tumor cells were loosely arranged with diffuse growth, vesicular nuclei, dyed cytoplasm, visible eosinophilic inclusions, and more nuclear fission.The results of immunohistochemical staining showed that the vimentin and epithelial membrane antigen were positive, whereas CK, CD99, CD 34, and S-100 were positive at different degrees. MyoD1, Desmin, and INI- 1 were negative. Conclusion : Rhabdoid tumor is rare and highly aggressive. It occurs mainly in the kidney and can also be found in other systems. The unique pathological form and im -munohistochemical staining observed on the tumor can be used as reference for diagnosis.