Coffin–Siris syndrome (CSS) is a rare genetic condition with characteristic musculoskeletal abnormalities and growth restrictions, coarse facial features, feeding abnormalities, systemic malformation, and developmental and cognitive delay.^1–3 Main anesthetic concerns should be difficult airway that tends to get worse with age, risk of aspiration, and periop pulmonary complications in uncooperative and mentally retarded patients. We report a case of 9-years-old male patient with CSS for dental extraction and restoration under general anesthesia.