The survival rate in congenital diaphragmatic hernia (CDH) with complex heart defects is low. Although the current consensus on the indications for surgical repair of CDH without heart defects has improved surgical outcomes, the surgical indication for CDH with complex heart defects remains unclear. Herein, we report the perioperative management of a patient with univentricular circulation who underwent CDH repair. Thus, patients with CDH complicated by univentricular anatomy may tolerate surgery depending on preserved respiratory function.