Neural tube defects (NTDs) are a group of congenital malformations of the central nervous system, which derive from an altered embryological development during neurulation. The different types of NTDs are closely related to their onset in the embryonic period; precocity is an index of greater severity of clinical manifestation. The myelomeningocele is one of the most common pathological conditions which is included into the spectrum of NTDs. It is characterized by the lack of fusion of the vertebral arches, resulting in eversion of the meninges and medullary content; it is the condition which clearly needs more assistance in the neonatal intensive care unit. The management of patients with NTDs is divided into different levels and particularly neonatologists have to monitor all the infants’ vital signs, to perform a careful neurological examination for the evaluation of motor and sphincterial functions and to recognize orthopedic abnormalities. Furthermore, concerning the open defects, which maintain a communication between the spinal cord and the outside, it is essential to prevent infections of the central nervous system through a broadspectrum antibiotic therapy. The diagnosis includes x-ray of the spine, which allows to locate the exact level of the vertebral defect and ultrasound scan of the region involved; also magnetic resonance imaging plays a very important diagnostic role. All this exams guarantees to every newborn affected by NTDs a comprehensive care approach, but also useful informations for diagnosis and prognosis.