We present a rare case of pulmonary atresia with intact septum associated with congenitally corrected transposition. The patient was a nondysmorphic female infant. Despite a right Blalock-Taussig shunt on day 13 of life and a balloon atrial septostomy on day 23, she remained ventilator dependent with poor systemic ventricular function. The patient died at the age of 160 days, and postmortem findings are presented. The literature on this condition is reviewed, and the possibility that the left-sided morphologic right ventricle could not sustain the systemic circulation is discussed. © 2006 Springer Science+Business Media, Inc.