Given improvements in the diagnosis and treatment of congenital heart disease, more patients with heart disease reach adolescence or adulthood and require noncardiac surgery. Since 1971, the Fontan operation has been widely used for the management of tricuspid atresia and a variety of univentricular heart malformations. Although the life expectancy of these patients has increased, few reports are available on their anaesthetic management when a noncardiac operation is indicated. We report on the anaesthetic management of a 40-years-old male patient scheduled for permanent pacemaker insertion who underwent a modified Fontan procedure at the age of 27 years for pulmonary stenosis, common atrioventricular cushion defect, and for corrected transposition of the great arteries