Tuberous sclerosis complex (TSC) is an autosomal dominant disorder with an incidence of approximately one in 6000. It arises from a genetic abnormality involving either the TSC1 gene on chromosome 9 or the TSC2 gene on chromosome
16. The protein product of TSC1 is hamartin and that of TSC2 is tuberin. In cells, hamartin and tuberin form a complex which inhibits the mammalian target
of rapamycin (mTOR), a central controller of cell growth and proliferation. Angiofibroma affects 70–80% of patients with TSC, typically on the face. We report a patient with TSC with recurrent life-threatening haemorrhage from both
kidneys due to extensive angiomyolipoma formation leading to bilateral nephrectomy and renal transplantation. Immunosuppressive treatment with rapamycin, a
specific mTOR inhibitor, initiated because of renal transplantation, reduced facial angiofibroma dramatically.