Tuberous Sclerosis Complex (TSC) is a multisystem disorder. Renal involvement in the form of angiomyolipoma or cyst is usually asymptomatic but can result in significant morbidity and End Stage Renal Failure (ESRF). Renal tranplantation has been perfomed successfully in individuals with TSC. We present an adult case of TSC with bilateral angiomyolipoma, who underwent renal transplantation successfully. Herein we describe a 22 years old man with ESRF secondary to TSC and bilateral angiomyolipoma. He was on maintenance hemodialysis for 4 years before he recieved cadaveric donor renal transplantation. His immunosuppresive medictions included steroids, azathioprine, tacrolimus and a short course of ATG. His graft functioned immediately with normalization of renal parameters within 48 hours postoperatively. Six months later he underwent an uneventful elective bilateral native kidneys nephrectomy. We concluded that renal transplantation is an effective form of Renal Replacement Theapy (RRT) in cases of TSC. Nephrectomy should be considered early in post renal transplant patients with angiomyolipoma. Laboratory data, radiological imaging and histopathological findings of this case will be discussed.