Renal tumours, in tuberous sclerosis, are mainly benign and correspond to angiomyolipomas, multiple and bilateral, sometimes enormous with an hemorrhagic risk, frequently associated with cysts of variable size and number and seldom with high metastatic carcinomas. Chronic renal failure is rare and related to focal glomerulosclerosis secondary to hyperfiltration due to nephronic reduction owing to surgery or to tumour invasion, in particular cysts. Data about 65 patients with tuberous sclerosis and chronic renal failure were collected thanks to a national survey. They were females in 63.1% of cases, with a mean age of 29, 2 years at the time of the diagnosis of tuberous sclerosis, revealed in near half of cases by renal involvement. There were angiomyolipomas in 23.1% of cases, cysts in 18.5%, both in 53.8% and malignancies in 12.3%. All but one of the 48 patients with end stage renal failure were treated by dialysis; 20 were transplanted, with good results.