Abstract
Increased salivation and contractions of the oropharyngeal muscles are frequently observed in Crisponi syndrome. This causes frequent recurrent lung infections. Anesthesia management can be challenging due to the frequent convulsions that occur during the intubation and extubation of the patient and subsequent cyanosis and hyperthermia attacks. Cold-induced sweating attacks may also occur due to the low operating room temperature. Hyperthermia attacks can lead to rhabdomyolysis and disseminated intravascular coagulation. Sudden deaths may occur in children with Crisponi syndrome. Hyperthermia, paroxysmal muscular contractions and trismus due to autonomic dysfunction are held responsible for sudden deaths.