A kidney transplantation was performed in July 1981 on a 29 year old woman who presented the signs of tuberous sclerosis and suffered from chronic renal failure. The frequency and the genetic transmission of Bourneville's disease are explained. The signs of the disease are exposed with special emphasis on the renal lesions. Only the patients with minor neurological symptoms can survive. These patients are able to develop chronic renal failure. This occurs either when the kidneys are destructed by renal cysts or tumors, or when a bilateral nephrectomy must be performed for bleeding or tumoral compression. A kidney transplantation can give them an opportunity to live almost normally. When nephrectomy is not performed, a regularly follow-up is necessary because the unknown future of the renal lesions in place.