Renal cell carcinoma in a 14-year-old girl with Von Hippel Lindau disease.

We report a new case of renal cancer in the context of Von Hippel Lindau (V HL) disease in a 14-year-old girl. To: our knowledge, this is the youngest case reported in the literature. VHL disease is now well known and its prog nosis is related to renal cancer, which occurs very early in the patient's life, requiring adequate surveillance of patients carrying the VHL gene to ensure early diagnosis of the cancer, screening of family members and treat ment, preferably conservative when permitted by the size and number of tumo urs, in these patients who are predisposed to developing other renal tumour s, in order to delay bilateral nephrectomy and the need for dialysis or eve n transplantation, to a maximum.