Isolated paraventricular frontal horn cysts are sometimes encountered on cranial ultrasound examinations of preterm neonates. The etiology and clinical significance of these lesions are unclear. The authors aimed to identify antenatal/intrapartum risk factors associated with the occurrence of these cysts and to assess developmental outcomes of preterm neonates with isolated frontal horn cysts. A retrospective cohort study with matched control design was used. A total of 28 cases were matched for gestation with 56 controls. No antenatal/intrapartum factors were associated with these cysts. At corrected age of 1 year, there was no difference in the mean general quotient between cases and controls (97.75 ± 17.28 vs 94.94 ± 9.86; P = .410). In all, 1 case and no controls had a diagnosis of cerebral palsy and 1 case and 3 controls had general quotients less than 80. The authors conclude that isolated paraventricular frontal horn cysts are benign, with no effect on neurodevelopment.