Abstract: Congenital myotonic dystrophy has been associated with
ventriculomegaly but hydrocephalus requiring a shunt in infancy is very
uncommon. Though there is a single case report of occlusive hydrocephalus
needing a shunt, there are no similar reports for communicating hydrocephalus,
which is more common in congenital myotonic dystrophy. We hereby report a
4-month-old baby who was diagnosed to have congenital myotonic dystrophy during
neonatal period and subsequently required a shunt because of communicating
hydrocephalus.