Von Hippel-Lindau disease is a rare autosomal dominant disease with incomplete penetrance and variable expression and is characterized by diffuse haemangioblastomas of the central nervous system and viscera. The majority of the central nervous system lesions are located in the cerebellum. This report describes the successful management with epidural anaesthesia of a woman with a term gestation, von Hippel-Lindau disease and cerebellar haemangioblastoma.