Tuberous sclerosis (TS) is an autosomal dominant multisystemic disease involving primarily the skin, the brain and the kidneys. Inspite of the kidney being involved in 40-80% of patients with this disease, the incidence of end stage renal disease is only about 1%. There are only 34 reported cases of successful renal transplantation in tuberous sclerosis patients with end stage renal disease. We report a case of successful renal transplantation in a patient of tuberous sclerosis with bilateral polycystic kidneys presenting with renal failure who also underwent bilateral native nephrectomies on follow up.