A case, 23 years old male, of Bourneville-Pringle phacomatosis (tuberous sclerosis complex) with the renal tumor was reported. Roentgenographic examination showed shadow suggesting left renal tumor, and nephrectomy was performed. The left Kidney, 250g, showed multiple tumor masses macroscopically and angiomyolipoma pathohistologically. In our case, it can be demonstrated mental retardation, epilepsy, adenoma sebaceum of the face, phacoma of the retina and multiple angiomyolipoma of the kidney. In familial history of this case, hereditary factor was unknown.
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