Teratoid</span></span></span><span><span><span><span style="font-family:""> </span></span></span></span><span><span><span><span style="font-family:""><span style="font-family:Verdana;">rhabdoid tumors are highly malignant, rare </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> aggressive. The prognosis is very poor, with a pejorative and rapidly lethal evolution. The objective of this study was to show </span><span style="font-family:Verdana;">diagnostic</span><span style="font-family:Verdana;"> and therapeutic approach through the report of four observations of </span><span style="font-family:Verdana;">rhabdoid</span><span style="font-family:Verdana;"> tumor of the kidney in children, treated in the oncology unit at the pediatric department CHU Hassan II Fez Morocco, collected </span><span style="font-family:Verdana;">over a period of</span><span style="font-family:Verdana;"> 10 years. The ages of the patients varied from 8 months and 5 and a half, with 3 girls and a boy. All </span></span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">children</span></span></span></span><span><span><span><span style="font-family:""> </span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">have </span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">abdominal distention with the discovery of a mass on clinical examination. </span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">The </span></span></span></span><span><span><span><span style="font-family:""><span style="font-family:Verdana;">patients were treated </span><span style="font-family:Verdana;"