Joubert syndrome is a rare autosomal recessive disorder of cerebellum that occurs in 1 of 100.000 live births. The syndrome is characterized by a distinctive cerebellar and brainstem malformation, hypotonic, developmental delays, either hyperpnea or apnea or atypical eye movements or both. Cognitive abilities are variable, ranging from severe to mild mental retardation. A magnetic resonance imaging (MRI) is the most common neuroimaging technique used to establish the diagnosis of Joubert syndrome. "Molar teeth" sign on MRI is spesific for Joubert Syndrome. There are limited reports on the anesthetic manegement of these patients. These patients may be sensitive to respiratory depression caused by anesthetics. Because of these reasons, anesthetic management of these patients need more attention. These patients are especially susceptible to N(2)O, opioids and neuromuscular blockers. Ventilation parameters must be monitored closely for at least 24 hours postoperatively.