Neural tube defects are one of the most common malformations in human beings. These occur due to failure of neural tube to close properly during its embryonic development. Its etiology has a strong genetic component. Prevalence varies according to geographical location, race, socioeconomic status, sex, maternal age, parity, nutritional deficiencies, occupational exposure, infectious diseases, hyperthermia, stress, gestational diabetes, etc.A proper history and physical examination helps arrive at a correct diagnosis.Untreated neural tube defects could induce premature closure of some cranial sutures, especially the coronal suture, by diminishing intracranial pressure and pulsations that are necessary to stimulate normal brain and skull growth. The upper jaw is usually too small in these syndromes resulting in crowded dentition. These patients therefore, might require orthodontic treatment and midface advancement.An orthodontist can easily come across minor malformations of sella during cephalometric evaluation and has an opportunity to participate in a growing field of clinical care.