Abstract: Objective: To investigate the clinical features, treatment and prognosis of tuberous sclerosis complicated with hemorrhage of bilateral renal angiomyolipomas. Methods: The clinical data of one case of tuberous sclerosis complicated with bilateral renal angiomyolipomas in our department was retrospectively analyzed, and the related literatures were reviewed. Results: The patient was a 27-year-old male. Due to sudden right lumbar and abdominal pain with hematuria, CT showed multiple renal angiomyolipomas and hemorrhage of right renal hamartoma. Selective renal artery embolization was performed in bilateral kidneys by stages. No rupture or bleeding was found after 6 months follow-up. Conclusions: Tuberous sclerosis complicated with bilateral renal hamartoma rupture and hemorrhage is rare and can rapidly progress to shock and multiple organ failure. Early identification, timely diagnosis and early renal artery embolization are the key points of treatment.