Background Malignant rhabdoid tumor of the kidney is the most aggressive childhood renal tumor. A preoperative diagnosis is critical in order to correctly establish a therapeutic strategy and a full metastatic workup. Case presentation We report on a 3-month-old case with fever, diarrhea, and abdominal distension treated surgically with adjuvant chemotherapy. The diagnosis was confirmed postoperatively. Relapse was quick, and the child died 5 months after surgical resection. Conclusions Rhabdoid renal tumor in young age is associated with a high mortality rate even with invasive strategies. Case reports and research are critical for evaluating existing protocols and improving prognosis. Diverse clinical trials are being conducted in the hopes of improving the prognosis of rhabdoid renal tumors.