Few patients with cyanotic congenital heart disease reach adulthood without a cardiac operation. The prognosis for ''unrepaired'' pulmonary atresia with ventricular septal defect is approximately 8% in the 1st decade of age. Consequently, the number of adults with this particular heart disease (unrepaired) who are expected to need a non-cardiac surgery is extremely low. General anesthesia may aggravate the preexisting right to left shunt and lead to persistent severe hypoxemia. The goal of anesthetic management should be to maintain intravascular volume. Systemic and pulmonary vascular resistance changes, such as might occur due to acidosis, hypothermia, hypercarbia or excessive airway pressures, should be avoided. Maintenance of preload, contractility and sinus rhythm is of major importance. The complex pathophysiology of such heart disease, in addition to the circumstances of emergency operation, exacerbate the total anesthetic risk. We present here a rare case of an acute appendectomy with successful outcome in an adult with pulmonary atresia and ventricular septal defect.