Rett syndrome (RS) is a progressive neurological disorder that almost exclusively affects females. It is characterized by severe mental retardation following a period of normal development during childhood. Classical features include autism, stereotypical hand movements, seizures, microcephaly and abnormal respiratory control. Anesthetic challenges in patients with RS include difficult airway, seizure management, positioning, increased sensitivity to sedatives, autonomic dysfunction, anesthesia-triggered episodes of apnoea, hypoxemia and sudden death. Here, we describe the successful anesthetic management of a 13-year-old patient with RS exhibiting most of these features who underwent laparoscopic SOS open ovarian cystectomy and hysterectomy.