Joubert syndrome is a rare genetic autosomal recessive disorder, which is estimated to occur in 80,000 to 100,000 live births. Brain magnetic resonance imaging (MRI) indicating the molar tooth sign can be an important indicator of Joubert syndrome. Prognosis depends on the severity and extent of respiratory disorder immediately after birth. Herein, we report the case of a five-year-old boy with Joubert syndrome, who visited the hospital with his parents. He was unable to chew because of toothache and tooth decay. Considering his poor clinical condition and inability to cooperate, dental procedures were performed under anesthesia at the hospital. Generally, these patients are sensitive to the respiratory effects of anesthetics, such as opiates and nitrous oxide; therefore, they should be avoided. In the present case, sevoflurane gas was used to induce general anesthesia. Advanced dental caries have been observed in previous cases, which might be attributed to dental hypoplasia and inability to observe dental and oral hygiene. Therefore, the patient's parents must be given the necessary instructions on the observance of orodental hygiene, and regular follow-ups are necessary for dental checkups and preventive measures.
Keywords: Hypoplasia; Joubert syndrome; Molar tooth sign.