Background: Joubert syndrome is a very rare condition with dismal prognosis. It is characterized by several abnormalities including molar tooth sign on MRI. When coupled with mega cisterna magna- a feature of the Dandy Walker syndrome- it is categorized as Joubert plus syndrome. Case report: A 16 month old male child with Joubert syndrome was referred to the Pediatric Dentistry Department Clinic, Faculty of Dentistry Alexandria University, complaining of severe tongue and lower lip injury due to self-mutilation. He required multiple teeth extractions under general anesthesia to prevent further tongue and lip mutilation. Conclusion: Joubert plus syndrome is a very rare occurring condition. Because self-mutilation is sometimes fatal, a treatment plan tailored to each patient's need is mandatory. A multidisciplinary approach is recommended.

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