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Tuberous sclerosis is a complex disorder which has multisystem involvement and varied clinical manifestations. Almost half of the patients have associated angiomyolipoma which contains fat, vascular and smooth muscle components. Spontaneous pseudoaneurysm formation is a complication of angiomyolipoma. Here we present a case of a female child presenting with hematuria who after thorough clinical and radiological investigations was diagnosed as a case of tuberous sclerosis with right intrarenal pseudoaneurysm. She was successfully treated with endovascular coil embolization.