Background: Transcatheter closure of congenital coronary artery fistulas (CCAFs) is an altative therapy to surgery; however, data regarding transcatheter closure for CCAF with a giant coronary artery aneurysm (CAA) in pediatric patients are still limited due to the rarity of the disease. We aimed to evaluate the efficacy and safety of transcatheter closure for CCAF with a giant CAA in a pediatric population at a single center. Methods: Medical records of pediatric patients (<18 years old) who underwent transcatheter closure of CCAF with a giant CAAbetween April 2007 and September 2016 at Guangdong Cardiovascular Institute (Guangdong, China) were reviewed. Results: Twelve patients (median age, 6.1 years; range, 1.9–11.0 years) underwent successful transcatheter closure procedures. One patient underwent closure at both the entry and exit points of the CAA, three patients underwent closure at the exit point of the CAA, and eight patients underwent closure at the entry point of the CAA. After a mean follow?up of 7.2 years (range, 0.5–9.8 years), one patient (with closure at the exit point of the CAA) underwent transcatheter re?intervention because of a significant residual shunt. She eventually underwent a surgical procedure due to aneurysm dilation after the second intervention. One patient experienced thrombus formation within the CAA after the procedure. Among those with closure at the entry point of the CAA, a mild?to?moderate residual shunt was detected in three patients. Conclusions: Transcatheter closure appears to be a safe and effective altative therapy for CCAF with a giant CAA in the pediatric population. Closure at the entry point of the CAA, and closure at both the entry and exit points when feasible, may reduce the risk of postinterventional complications.