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Tuberous sclerosis is a rare genetic disease with multiple organ involvement. Renal involvement is manifested by the presence of angiomyolipomas and cysts. Angiomyolipomas due to the progressive increase in size over time can complicate by bleeding that can lead to severe retroperitoneal hemorrhages. Hence, it is important to perform an active surveillance of the masses and to institute therapeutic attempts in order to manage or prevent any complication. We present three clinical cases of three women with various clinical presentations and treated in different ways. Recently, the employment of mTOR inhibitors such as Everolimus has been shown therapeutic efficacy in this disease, especially in terms of a dimensional reduction of renal angiomyolipomas., (Copyright by Società Italiana di Nefrologia SIN, Rome, Italy.)