We present the anesthetic management of a 10-year-old boy with Escobar syndrome scheduled for left tympanoplasty. This syndrome may pose many problems to anesthesiologist especially regarding management of airways, difficult venous access, psychological problems because of multiples surgeries and probably association with malignant hyperthermia. Considering all of this we propose the use of LMA and TIVA for suitable operations in children with Escobar syndrome.