Background: Refractory hemorrhage is a fatal complication during peri-operative period of congenital heart disease (CHD) in children, which has great influence on the prognosis of patients. Recombinant human coagulation factor VIIa (rFVIIa) has been widely used to treat refractory bleeding, but there is no clear randomized controlled trial or guidance to guide clinical application. It still varies from center to center about the dosage, therapeutic efficacy and adverse effects of rFVIIa in refractory bleeding treatment. Objective: This article aims to address the role of rFVIIa in refractory bleeding as to guide clinical applications of rFVIIa. Content: This article reviews the indications, dosage, efficacy and adverse reactions of rFVIIa during the perioperative period of CHD. Trend: As more studies carry out, the use of rFVIIa will be more standard and safe.