The management of childbirth, including epidural anaesthesia for Caesarean section is described in a patient with von Hippel-Lindau disease. The reasons for the choice of epidural anaesthesia are explained and the anaesthetic significance of the disease is discussed.

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.