To determine whether seizures are a reliable sign of increased intraventricular pressure in children with shunt-dependent hydrocephalus and meningomyelocele, we performed a retrospective chart review of 346 patients with meningomyelocele and shunt-dependent hydrocephalus. Fifty-one patients had seizures. Seizure episodes were investigated to determine whether they were temporally associated with shunt dysfunction. Episodes of actual or presumed shunt dysfunction were analyzed as to presenting symptoms, ventriculogram, computed tomography scan, and shunt film results. These 51 patients had 129 admissions for possible shunt dysfunction. One hundred one admissions were due to increased intraventricular pressure; nine (9%) of these, had a seizure as one of the presenting symptoms. All of the nine episodes had other common presenting symptoms of shunt dysfunction, such as headache, vomiting, lethargy, or respiratory compromise. We conclude that seizures alone are an inadequate predictor of shunt dysfunction in children with meningomyelocele, but can be seen as one of the presenting symptoms.