Most neonatal seizures in preterm newborns are of acute symptomatic origin with a prevalence higher than in full-term infants. To date, recommendations for management of seizures in preterm newborns are scarce and do not differ from those in full-term newborns. Mortality in preterm newborns with seizures has significantly declined over the last decades, from figures of 84%–94% in the 1970s and 1980s to 22%–45% in the last years. However, mortality is significantly higher in those with a birth weight < 1000 g and a gestational age < 28 weeks. Seizures are a strong predictor of unfavorable outcomes, including not only cerebral palsy, epilepsy, and intellectual disability, but also vision, hearing impairment, and microcephaly. The majority of patients with developmental delay are severely affected and this is usually associated with cerebral palsy. Furthermore, the incidence of epilepsy after neonatal seizures seems to be lower in preterm than in full-term infants but the risk is approximately 40 times greater than in the general population. Clinical studies cannot disentangle the specific and independent contributions of seizure-induced functional changes and the role of etiology and brain damage severity in determining the long-term outcomes in these newborns.