Abstract

Neurofibromatosis (NF) is rare in our country; skin, nervous system, or both holds together, is a heterogeneous disease hereditary. NF type I (Von Recklinghausen disease) 2500-3000 births constituting a monitoring and genetic susceptibility to the most common neoplasms. It is a neurocutaneous disease. In association with intrahepatic cholangiocarcinoma NF type it is rare. 36 Years intrahepatic diffuse neurofibromas on the whole body of our patients who were followed by cholangiocarcinoma diagnosed; back "cafe au lait" spots on the iris and "Lisch nodules" We've found. Thus our diagnosis of NF type 1 cases, we aim to share because of its rarity.