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Angiomyolipoma is one of the renal benign neoplasms. The most of the angiomyolipomas are asymptomatic and found incidentally with ultrasound. They are more prevalent in patients with tuberous sclerosis. It is very important to make differential diagnosis from other renal neoplasm such as renal cell carcinoma. Growth rate is higher among pregnant women suggest that the hormones may play a role in the stimulation of angiomyolipoma. The most common serious presentation is rupture and hemorrhage, and sometimes it can lead to shock. Adequate diagnosis, treatment, and follow-up are very important in the face of renal angiomyolipoma.

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