A patient with typical manifestations of von Hippel-Lindau disease is presented. In this 44 year-old man, cerebellar hemangioblastoma was found initially and he was operated upon at the age of 25. This was followed by retinal angiomas treated by cryocoagulation and, recently, by paresthesia of both legs and frequent shooting pains over the lower abdomen. A computed tomography disclosed multiple small cysts of the pancreas, bilateral multicentric renal carcinomas and a spinal angioma in the lumbar portion, confirmed by magnetic resonance imaging, and consistent with neurogenic manifestations. Multiple enucleations of the renal cell carcinomas were carried out, most of which were histologically well differentiated adenocarcinomas. There was no family cluster. Six months postoperative, the patient is doing well.