Bardet-Biedl syndrome is a rare autosomal recessive genetic disorder that affects many organ systems. In cases of Bardet-Biedl syndrome, since the risk of developing inspiratory, cardiovascular, and metabolic problems is high, endotracheal intubation and anesthesia management are difficult. In this report, we present our experience in the management of anesthesia during endoscopic sinus surgery that was performed for the first time on a 6-year-old pediatric patient diagnosed with Bardet-Biedl syndrome.