[Von Hippel Lindau disease: about one case]

Von Hippel Lindau disease is an autosomal dominant inherited disorder secondary to a mutation of the VHL gene, localisated on the chromosome 3, which is a tumor suppressor gene. Tumoral manifestations include hemangioblastomas, renal cysts and/or tumors, pancreatic cysts and/or neuroendocrine tumors, pheochromocytomas and endolymphatic sac tumors. Early diagnosis is necessary to allow for a regular follow up of the patient and his descendants