Our aim is to present a case of a rare disorder, such as Joubert syndrome (JS) which required oral surgical intervention under general anesthesia at a very young age to help in achieving normal developmental milestones.

Ciliopathies are an emerging class of diseases of which JS is a significant member. There have been very few cases of JS with its distinguishing features which have been reported in recent literature.

We herewith present a case of JS who reported to our unit with a complaint of speech abnormality. Intraoral examination revealed a tongue-tie which was planned for surgical release. As any drugs that interfere with respiratory depression have to be used with utmost care, adequate precautions were taken in this case and the patient was treated under general anesthesia. The tongue-tie was released and associated hamartomatous outgrowths from the floor of the mouth were also excised. Appropriate postoperative care was taken and the patient was discharged.

The patient treated by us did not reveal any significant intraoperative or postoperative complications. Treatment of cases of JS required a team effort of multiple dental and medical specialists. Long-term follow-up of such patients is required so that they achieve normal chronological development.

Joubert syndrome being a rare disorder requires special anesthetic care during any surgical procedure, especially that involving the oral cavity. The need and timing of surgical intervention in such cases should be carefully assessed and appropriate precautions should be taken.

Gurjar V, Gurjar M, Pattanshetti C, Sankeshwari B. Lingual Frenectomy in Joubert Syndrome. J Contemp Dent Pract 2017;18(8):728-731.