The renal angiomyolipoma is a rare, benign, no epithelial neoplasm. It is frequently associated with tuberous sclerosis, above all in young patients. It may be under an asymptomatic form, especially if it deals of a small volume neoplasm, and sometimes it becomes evident only when it occurs an haemorrhagic complication. When we have a sure diagnosis and a small tumor, the treatment is preservative; on the contrary it is surgical either for big symptomatic neoplasms or haemorrhagic complications. The authors report a clinical case of renal angiomyolipoma in a pregnant patient, who had an haemorrhagic complication and was treated with nephrectomy.