Introduction: Angiomyolipoma is a benign renal tumor, possibly derived from epithelioid cells surrounding blood vessels. The tumor consists of multiple vessels, smooth muscle and adipose tissue. Angiomyolipoma constitutes less than 10% of renal tumors, with autopsy series about 0.3% and ultrasound screened populations around 0.13%. Angiomyolipoma is more common in females, rare before puberty, suggesting a potential hormonal influence. Case Report: We are reporting a case of multiple, bilateral angiomyolipomas in a young lady with tuberous sclerosis presented for the first time with acute spontaneous retroperitoneal bleeding and hypovolemic shock. CT scan was diagnostic and because of high risk of bleeding, biopsy was not recommended for this case. The mainstay of treatment was selective embolization which provides the nephron-sparing approach necessary to preserve renal function and overall quality of life. Conclusion: Preservation of renal function and overall quality of life are the main target in the management of this urologic dilemma and whether conservative medical treatment followed by intervention is superior to emergency intervention (embolization; open or laparoscopic/robotic partial nephrectomy) remains the sole question to be answered in the future.