[Bilateral renal adenocarcinoma: relationship with phakomatosis]

Von Hippel-Lindau disease is a very uncommon finding in our environment. A case of an incidental finding of that entity expressed through retinal angioma, bilateral renal cysts and bilateral renal carcinoma is described. A surgical course was followed performing a radical left nephrectomy, given the large size of the tumour, and right lower partial nephrectomy, as well as a review of the renal cysts considering the big tendency they have to degenerate in the disease.