A case of spontaneous, but not contemporary rupture of both kidneys in a 42-year-old man with tuberous sclerosis is described. Despite the high incidence of renal involvement, chronic renal failure due to replacement and compression of renal tissue by hamartomas is rare. We reviewed 25 cases in the literature and found only 1 other case which necessitated maintenance hemodialytic treatment for kidney rupture. It is suggested that early diagnosis and conservative partial nephrectomy could prevent or delay the onset of chronic renal failure, malignant transformation and dramatic rupture of hamartomas.