Different minimally invasive methods have been proposed to treat renal manifestation of tuberous sclerosis complex (TSC), in order to preserve kidney parenchyma and avoid nephrectomy. The case of a boy with bleeding giant angiomyolipoma treated by selective arterial embolization is discussed. The extensive renal lesions resulted significantly decreased, with resolution of arterial hypertension and abdominal symptoms. Differential renal function increased from 26.6% to 32.6%. Renal involvement in TSC requires careful parenchymal-sparing procedures. Selective embolization of the main renal lesions should be considered as first line therapy.