Purpose: The objectives of this study were as follows: to present the course and outcome of pregnancies complicated with neural tube defects, determine the association between prenatal ultrasound diagnoses, and definitive diagnoses after autopsy. Matherial and Methods: The survey was designed as a retrospective study and included 24 pregnant women who were attending a regular ultrasound examinations at the Department of Gynecology and Obstetrics, Clinical Center of Serbia, or patients who were referred from other institutions in Serbia. Results: Neural tube defects are divided into five subgroups: spina bifida, meningocele, myelomeningocele, acranius, and anencephaly. The most frequent in the present study was spina bifida with 67%. All pregnancies complicated with neural tube defects were terminated. Conclusion: Their clinical severity and uncertain cause make them priorities for further research, whether to better target primary preventive measures, to improve in-utero surgery for prenatal repair, or to identify the causative genes to provide an objective basis for individual genetic counselling.