BACKGROUND Joubert syndrome is a rare autosomal recessive disorder first described in 1969, with an estimated prevalence of 1 in 100 000. Joubert syndrome is characterized by partial or complete agenesis of the cerebellar vermis - the structure that connects both parts of the cerebellum. This results in the main clinical symptoms, such as muscular hypotonia, ataxia, mental retardation, abnormal eye movements, and a central apnea breathing pattern. Joubert syndrome can combine neurological signs with variable multi-organ involvement, mainly of the retina, kidneys, liver, and musculoskeletal system. CASE REPORT A 13-year-old boy presenting with recurrent otitis media, fever, respiratory infections, and tonsillar hyperplasia needed surgery. At the otorhinolaryngology outpatient clinic, the indication for surgical paracentesis, adenoidectomy, and tonsillectomy under general anesthesia (first in his life) was set. We performed a total intravenous anesthesia (TIVA) using propofol (described as safe) and remifentanil (organ-independent metabolism) without any side-effects. For postoperative pain therapy we used metamizole instead of paracetamol in order to avoid liver injury. CONCLUSIONS Due to the possible facial dysmorphism we recommend a critical evaluation of the airway to assess a potential difficult airway preoperatively. Our case underlines that TIVA, with the medications used in this case, is safe. We refrained from premedication in order not to trigger central apnea. For safety reasons, all preparatory procedures were carried out in the recovery room under monitor surveillance and with audio-visual distraction for the patient in order to reduce the stress level. For postoperative pain therapy, we recommend the use of metamizole.