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A 24-year-old lady presented with left flank pain of 3 months duration. She had stigmata of tuberous sclerosis complex in the form of angiofibromas on face, ash-leaf macules on back and right upper limb and shagreen patches over back. Computed tomography scan of the abdomen showed 6.5 cm × 5.0 cm × 4.4 cm lobulated intensely enhancing exophytic mass lesion in mid pole of left kidney with significant para-aortic lymphadenopathy with no evidence of fat in the mass. She underwent radical left nephrectomy with a provisional diagnosis of renal cell carcinoma. Histopathological examination showed multicenteric angiomyolipoma involving kidney and para-aortic lymph nodes. This case report underscores the need for further research to differentiate fat-poor angiomyolipoma and lymphadenopathy from renal cell carcinoma.