Background: Abnormal respiratory control has been clearly documented in infants and children with Joubert Syndrome (JS) by polygraphic recordings, characterized by episodes of apnea, tachypnea, and/or hyperpnea and the risk of recurrence of these episodes increase probably in perioperative period. In those cases, the choice of anesthesia technique and postoperative pain protocol, constitute a challenge for anesthesiologists. Case: We describe a case of successful ultrasound-guided Caudal Epidural Block (CEB) for children above 6 years with Joubert Syndrome undergoing bilateral testicular ectopy and circumcision avoiding opioid-use. Conclusions: Despite the difficulty to achieve CEB in child above 6 years, the ultrasound-guided can increase the success providing effective analgesia in-patient with a high-risk of respiratory failure as child with JS.