Joubert Syndrome is an autosomal recessive disorder that is characterized by the cerebellar vermis defect which is ranged from hypoplasia to agenesis, tachipnea and apnea attacks in neonatal period, severe physichomotor retardation, jumping eye movements, protrusion of the tongue, ataxia and hypotonia. In addition to these symptoms, some different abnormalities and disorders were also described. In this case report, we aimed to report the results of the anesthesia performed under the emergency condition, for the patient with Joubert Syndrome who developed acute renal failure.