Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder with vari
able penetrance. It is mainly characterized by haemangioblastomas of the re
tina and central nervous system. Because of physiological effects of uterin
e contractions, labour and spontaneous vaginal delivery increase the risk o
f disrupting central nervous system haemangiblastomas. We report the case o
f a 28-year-old woman with an history of VHL disease who had an epidural an
aesthesia for labour and for Caesarean section performed because of failure
to progress and of fetal distress. The overall maternal and neonatal outco
mes were excellent. (C) 2001 Editions scientifiques et medicales Elsevier S
AS.