Neural tube defects are common congenital malformations that could be apparent at birth or manifested in later stages of life. Spina bifida is a type of neural tube defect that results from failure of neural tube closure during the first month in utero. Predisposition to dental caries, latex allergy, and craniosynostosis causing maxillary deficiency are some of the key problems in spina bifida. In this case, we would like to present the root canal treatment we performed in a patient with spina bifida who presented to our clinic with pain in the right lower molar tooth and developmental root shape anomaly in the mandibular first molar teeth.