Tuberous sclerosis is a rare hereditary disease which appears immediately after birth of during the second and third year of life. It is a multiorgan disorder characterized by convulsions, mental retardation and focal angiofibromyoma. The main findings are brain lesions including tuberous and astrocytes hamarthomas by which this disease was named. Renal alterations are angiofibromyolipoma and cysts, which are present in 40-80% of patients. The diagnosis is based on clinical, radiological and histological findings. This disease has a progressive course and fatal outcome. The therapy is symptomatic and surgical. The aim of this paper was to present this rare disease, which occurred in this patient during fourth year of life. Besides brain changes the patient also has extensive morphological renal alterations and renal failure. She died in 40th year of life due to multiorgan dysfunction.