Five preterm infants who had episodes of conjugate downward gaze 2-3 months after birth were evaluated. Three were extremely preterm, born at 22-25 weeks gestation, and 2 were twins, born at 28 weeks gestation. In all patients, multiple, brief episodes of downward gaze occurred daily, with each lasting for several seconds. In 3 infants, downward gaze occurred more often with stimulation, while in another it occurred more frequently prior to feeding. There was never an associated change in vital signs, level of consciousness, muscle tone, or focal neurologic deficit. All infants had bronchopulmonary dysplasia. Neuroimaging studies were normal in 3 patients and demonstrated nonspecific abnormalities in 2. At follow-up (6 1/2-34 months corrected age), intermittent downward gaze had completely resolved in 3 infants and was decreased in frequency and severity in the youngest 2. Four infants had normal development and neurologic examinations, while 1 child demonstrated mildly increased asymmetric muscle tone with normal development. Intermittent tonic downward gaze can be seen in extremely preterm infants who subsequently have normal development, and may represent a maturational delay in upward gaze reflex systems.