The authors describe a paradigmatic case of a large renal angiomyolipoma not associated with tuberous sclerosis. The lesion was discovered as an incidental finding during abdominal ultrasound for other pathology. Owing to the extent of the lesion and the appreciable risk of spontaneous rupture and bleeding, we opted for surgical treatment. The impossibility of dissecting the angiomyolipoma from the right kidney obliged us to perform a nephrectomy. The clinical interest of angiomyolipoma is in its rapid growth, the difficulty of distinguishing it from malignant lesions, the association with tuberous sclerosis, the presence of numerous aspecific symptoms and the difficulty of establishing the correct diagnosis and treatment strategy.

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