Closure of residual ventricular septal defect with an occluder is traditionally performed by a percutaneous transcatheter approach under radiographic guidance. However, this procedure may be of limited use in cases with unusually shaped defects and in patients with low body weight. Here, we report minimally invasive surgical device closure of a 6 mm residual ventricular septal defect under transesophageal echocardiographic guidance, in a patient weighing 10 kg that had previously undergone surgical correction of a double outlet right ventricle. The defect was positioned in the suture line between the Gore-Tex vascular graft and the remnant ventricular septum, and was unusual in that it formed a 135� angle with the Gore-Tex graft. The defect was closed successfully with a 10 mm asymmetric occluder. To the best of our knowledge, this is the first report of transesophageal echocardiography-guided minimally invasive surgical device closure of an unusually shaped residual ventricular septal defect after surgical correction of a double-outlet right ventricle.