Central nervous system (CNS) tumors, including brain and spinal cord tumors, are the most common solid tumors of childhood. Within the neonatal population, however, CNS tumors are relatively rare. These often carry a dismal prognosis in part due to the limited therapeutic options available for newborns and the unique biology of these tumors compared with those seen in older infants and children. This article reviews neonatal CNS tumors, specifically their clinical presentation, imaging findings, treatment, prognosis, and associated genetic syndromes. The unique psychosocial and emotional challenges facing clinicians and families are discussed as well.