RESUMO
Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem neurocutaneous genetic condition. It is characterized by TSC-associated neuropsychiatric disorders, epilepsy, tumors, and angiomyolipoma in multiple organs, such as the skin, lungs, and kidneys. TSC is also associated with the development of aneurysms of the medium and large arteries, including the renal arteries. This condition will usually be diagnosed early in life, and active surveillance is required of tumor and aneurysm growth to prevent life-threatening events. We have presented the case of a 41-year-old patient with TSC that had not been previously diagnosed. The patient had presented with retroperitoneal hematoma secondary to the rupture of two left renal artery branch aneurysms that had likely developed within the angiomyolipoma.