We present a case report about percutaneous closure of a congenital Gerbode defect using Nit-Occlud® Lê VSD coil. The patient was referred to our hospital with a diagnosis of ventricular septal defect (VSD) and severe pulmonary arterial hypertension. But transthoracic echocardiography revealed a communication between the left ventricle (LV) and the right atrial (RA), called Gerbode defect. Catheterization confirmed the shunt from the LV to the RA. We successfully closed the defect with a VSD coil. After uneventful 6 mo follow-up, the patient was out of dyspnea, the symptom urged him to have medical attention. This case report is to discuss the diagnosis and percutaneous treatment approach for this rare congenital heart disease.