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Background: A renal angiomyolipoma is a mixed mesenchymal benign tumor composed of smooth muscle, adipose tissue, and blood vessels. Malignant transformation of angiomyolipomas is anecdotal. To our knowledge, only 6 cases have been reported, and 4 of the patients had tuberous sclerosis complex diagnosed. Case Report: We present the case of a 29-year-old male with tuberous sclerosis complex who arrived at the emergency room with gross hematuria and a painful right-sided abdominal mass. Imaging studies revealed active bleeding from a giant angiomyolipoma. An emergency nephrectomy was performed. Histopathology evaluation revealed an angiomyolipoma with a focal lesion and clear cell renal carcinoma within the tumor. Conclusion: Limited evidence is available to dictate management of collision tumors of the kidney in the scenario of tuberous sclerosis complex, so a multidisciplinary approach that includes urology, oncology, genetics, and nephrology intervention needs to be considered. No standardized follow-up modality has been established for angiomyolipomas, so patients should be placed under active surveillance, similar to that carried out in cases of renal cell carcinoma.

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