Objective: To describe an atypical presentation of von Hippel-Lindau disease.
Material and methods: We present the clinical, diagnostic and treatment of the urological signs of this disease, which has overcoat a neurological management, in a young man with familiar history of it.
Conclusions: The basic knowledge of the von Hippel-Lindau disease is important for the urologist because the urological signs of it, are common and they make up one of the most important causes of morbility and mortality.