Neural tube defects are common congenital malformations that could be apparent at birth or manifested in later stages of life. Morbidity is high in anencephaly, whereas in spina bifida, there are neurological and motor disorders. These defects deserve paramount importance in clinical dentistry. Latex allergy, dental caries, difficulty in mouth opening, and sitting in a dental chair are common problems. There is a high risk of anaphylactic response during anaesthesia. There could be associated craniosynostosis causing maxillary deficiency, and malformed sella turcica might be seen. An association of the defects has been linked with orofacial clefts and Down syndrome.