Renal rhabdoid tumor is a rare and extremely aggressive tumor that results in multiple and early metastases. Through our case, we recall its diagnostic peculiarities and its therapeutic modalities. Case presentation: We report the case of a 5-year-old girl with a rhabdoid tumor in the left kidney with multiple retroperitoneal lymphadenopathies. Discussion: Kidney rhabdoid tumor was originally described as a "Rhabdomyosarcomatoid" variant of Wilms tumor due to the resemblance of cells to rhabdomyoblasts. Currently, this type of tumor is recognized as a distinct and unique malignant renal tumor and it usually affects children under 2 years of age. Sampling of tumor tissue is mandatory for diagnosis of Malignant rhabdoid tumor of the kidney (MRTK), either based on nephrectomy, baseline biopsy, or autopsy samples. There is no established therapeutic standard due to the rarity of cases. However, so far, surgery is considered to be the first choice of treatment whenever possible. Conclusion: In order to be able to discuss possible treatment options, it is important to recognize this tumor entity and its specificities

Keywords : Chromosome 22, GFA NEPHRO 2005, Radiotherapy, Rhabdoide