Von Recklinghausen disease and hepatic neurofibromatosis

Von Recklinghausen's neurofibromatosis is one of the most common autosomal dominant disease with an estimated frecuency of 1:3000 live births. Characteristic lesions include cafe-au-lait spots and neurofibromas following the path of peripheral nerves. Liver involvement by neurofibromatosis is rare and very few cases have been reported. We present a case of a young man with Von Recklinhausen's disease and hepatic neurofibromatosis with multiple caf-au-lait spots, cutaneous neurofibromas, short stature and osseous lesions and compare the clinical, radiological, surgical and anatomopathological findings with others describe previously in the literature.