A pregnancy in a patient with ventriculoperitoneal (VP) shunt was recently managed at the authors's institution. Review of the literature showed only six previous case reports. The management of this uncommon neurosurgical condition in pregnancy is presented along with a review of the literature. We conclude that pregnancy in a patient with a VP shunt for maternal hydrocephalus, generally has a normal outcome and that the function of the shunt is unaffected by pregnancy.