Tetralogy of Fallot with atresia of the pulmonary artery (ToF-PA) is a rare congenital cardiac malformation. The life expectancy of affected canine individuals is short: in fact, only puppies or young adults with ToF-PA are described in literature. Typically, these patients require general anaesthesia for diagnostic procedures, and are considered at high risk for anaesthesia-related complications. The present report describes successful anaesthesia for dental surgery of a 12-year-old dog affected by ToF-PA and an extracardiac shunting vessel connecting aorta and pulmonary artery. The echocardiographic findings are described and the anaesthetic protocol and the complications encountered during anaesthesia are discussed.