Tuberous sclerosis with cystic renal disease and multifocal renal cell carcinoma in a baby girl

An infant, in whom the prenatal diagnosis of tuberous sclerosis complex was made, presented with extreme bilateral nephromegaly owing to diffuse cystic changes. Histology of the resected non-functioning left kidney revealed, in addition to the characteristic cysts, two foci of renal cell carcinoma not visible on US or MRI. This infant is exceptional given the extensive cystic transformation of both kidneys and the presence of malignant lesions at this young age.