Patients with spina bifida have multiple ophthalmologic problems, many of which are preventable. Most of the problems are related to the hydrocephalus, which is caused by the coexisting Arnold-Chiari malformation. When patients are treated for hydrocephalus, and comprehensive eye care is available, 94% of the patients will have 6/12 visual acuity or better. Strabismus is common but it responds well to medical and surgical treatment. Children with spina bifida should have frequent examinations by an ophthalmologist who is familiar with the diagnosis and management of the defects recorded in this study.