Two cases of 14 and 11 years old children with tuberous sclerosis were admitted to Department of Paediatric Surgery in Wrocław and Department of Paediatric Hematology and Oncology in Lublin because of renal tumors. Radical nephrectomy was performed in both cases. Histopathological diagnosis revealed malignant rhabdoid tumor (MRT) in a 14 years old boy and benign angiomyolipoma in the latter case. The authors stress the rarity of MRT in older children and successful treatment due to nephrectomy and maintenance chemotherapy.