| Summary: | Renal angiomyolipomas (AMLs) are the most common benign renal tumors encountered, and composed of 3 components: mature adipose tissues, smooth muscles, and blood vessels. Mostly asymptomatic and discovered incidentally, the classic type of AMLs rarely extend to involve great vessels. Radiological confirmation of such lesions is paramount for diagnosis and planned intervention. Management of AMLs is based on clinical presentation and varies from active surveillance to invasive surgical interventions. A case of sizeable classic AML with extension to inferior vena cava is presented here, with successful tumor resection performed after complete liver mobilization. A literature review and a summary of similar cases are also presented. A multidisciplinary approach is required for proper and precise radiological diagnosis to achieve an adequate surgical resection, which might sometimes be complicated and complex, as in this current case. Keywords: Angiomyolipoma, Renal, Tuberous sclerosis complex, Fat emboli, Surgery, Liver mobilization
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