Grade IV intraventricular hemorrhage (IVH) is a severe complication of premature birth. We retrospectively performed serial exams on 13 infants with grade IV IVH and compared their ocular findings to 11 infants matched by gestational age and birth weight without an IVH. All children in the study were initially examined in the neonatal unit within 8 weeks after birth. Subsequent periodic eye exams were performed until 3 to 7 years of age. Of the 13 grade IV IVHs, there was a 92% incidence of ocular abnormalities, compared to a 73% incidence in the premature group without IVH. The children with grade IV hemorrhages had a statistically significant increased incidence of severe eye disease with resultant visual loss (38%) compared to the non-IVH group. Loss of vision was due to optic atrophy and retinopathy of prematurity. Grade IV IVH children are at high risk for severe eye disease and should have early and frequent ophthalmologic exams.