Tuberous sclerosis, caused by germline mutations in the TSC1 or TSC2 genes, is 
associated with aberrant upregulation of the mammalian target of rapamycin 
(mTOR) signalling pathway, resulting in growth of tumours, including renal 
angiomyolipomas (AMLs). AMLs may cause hypertension, renal failure and 
spontaneous life-threatening haemorrhage. Previously, invasive interventions 
were required to treat AMLs. More recently, mTOR inhibitors have been used as 
molecularly targeted treatment to treat AMLs. We present here the case of a 
paediatric patient with TSC in whom sirolimus has been used successfully to halt 
growth of renal AMLs.