• We undertook a study of premature infants with cyanotic congenital heart disease to determine whether these infants develop retinopathy of prematurity despite a persistent hypoxemic state. Using the computerized registry of the neonatal intensive care unit of Vanderbilt University Medical Center, Nashville, Tenn, we identified six premature infants (<37 weeks' gestational age, with birth weights of 1100 to 2050 g) with cyanotic congenital heart disease who survived the neonatal period and underwent ophthalmologic evaluation. Review of their charts revealed that three of six infants developed retinopathy of prematurity (two had grade 1 and one had grade 3 disease), but none required treatment. Our data support the findings of other investigators that elevated arterial oxygen tension is not the sole factor leading to the development of retinopathy of prematurity. Premature infants with cyanotic congenital heart disease can develop retinopathy of prematurity despite persistent hypoxemia. Cyanotic premature infants should be screened for retinopathy of prematurity with the same thoroughness as other premature infants.