This research aimed to examine the clinical and prognostic indications of paediatric malignant rhabdoid tumors of the kidney (MRTK) and to expand the understanding of the occurrence and progression of MRTK. From July 2014 to September 2021, all cases were confirmed by postoperative pathology investigation. Among the 42 patients were 25 boys and 17 females, with a median age of 10 (1–84) months. Abdominal mass or hematuria was the predominant clinical symptom. Preoperative chemotherapy was performed in 9 patients (VC). The tumor phases were stage I-IV. Preoperative metastases were observed in 9 patients; lung was the most common site. Postoperative patients underwent conventional chemotherapy, including VDACE regimen and UH-1 regimen. Among the 42 children in this group, survival at follow-up in this study was 26.2% (11/42). Preoperative anemia Univariate analysis identified preoperative anemia had a shorter survival time. In addition, tumor-related characteristics had a substantial impact on survival, with inadequate tumor excision, lymph node metastases, and stage III-IV had a decreased survival rate. The impact of postoperative variables on survival included postoperative complications that had a decreased survival rate. The children were younger than 12 months, had preoperative metastases, and no chemotherapy was performed after surgery was an independent risk factor for the prognosis of MRTK. The primary clinical signs about MRTK were abdominal mass and hematuria. In this case, preoperative treatment did not appreciably enhance the patient’s prognosis. The success percentage of a patient’s operation can be greatly improved by chemotherapy administered afterward. Diagnostics require a combination of clinical signs, imaging, histology, immunohistochemistry, and other forms of expert opinion. A poor outcome was seen in patients younger than 12 months, those with preoperative metastases, and those who did not have postoperative chemotherapy.