Idiopathic intracranial hypertension is more common among women of reproductive age and is often encountered in pregnancy, either pre-existing and exacerbated by pregnancy-associated weight gain and hormonal changes or arising de novo. We report the case of a 33-year-old woman with progressive visual loss and intractable headache from 20 weeks’ gestation requiring ventriculoperitoneal shunting during pregnancy. The risk of permanent maternal vision loss raises complex management dilemmas, when this must be balanced with the fetal and neonatal risks of treatment and possible premature delivery.