[Renal carcinoma and von Hippel-Lindau disease].

Von Hippel Lindau disease is a highly uncommon autosomic dominant condition characterised by the presence of cerebellar hemangioblastomas, retina angioma, pancreas, kidney and epididymal cysts, and renal cells carcinoma. This article describes the case report of a male patient with Von Hippel Lindau disease which presented as a jaundice secondary to biliary obstruction due to pancreatic cystic mass. After urological examination, bilateral cystic lesions and right renal solid lesion were detected requiring surgical treatment. A review is made of the diagnostic and therapeutic aspects, highlighting the significance of early diagnosis and treatment.