Abstract: Objective: To study the clinical features of tuberous sclerosis accompanying large renal angiomyolipoma and treatment of hemorrhage due to spontaneous renal rupture. Methods: The clinical data about 3 female cases of tuberous sclerosis accompanying bilateral and multiple renal angiomyolipomas were reported.Their age ranged 16-26 years.All the cases had typical facial angiofibroma and mental retardation.Of them,2 were admitted to hospital for severe abdominal distending pain and shock,1 was admitted to hospital for abdominal distention and epilepsy.Cranticocerebral CT revealed typical calcified cortical tuber and /or subependymal nodules,but no etrarenal organ angioleiomyoma in the 3 cases. Results: One case underwent renal tumor embolization with DSA,1 case underwent nephrectomy,1 case received nephrectomy after anti-epilepsy treatment.The 3 cases recoverd well and were discharged home. Conclusion: Tuberous sclerosis accompanying severe renal angiomyolipoma,suspicious canceration and potential bleeding should be treated radically removed.Renal artery embolization is the first choice for bleeding due to renal rupture.