A 6-year-old white boy was diagnosed to have a large aortopulmonary collateral artery during preoperative evaluation for adenoidectomy. He had a history of recurrent respiratory tract infection, symptoms of gastroesophageal reflux and scoliosis. Saturation was normal and no abnormal auscultation findings were noted. His weight was 20.7kg (25–50 percentile) and height 119cm (75 percentile). Prenatal exposure to carbamazepine because of maternal epilepsy was reported. He was delivered at term (birth weight 3100g). He underwent ventricular and atrial septal defects correction in the perinatal period. Since birth facial malformations (long philtrum, micrognathia, epicanthic fold) and paralysis of facial muscles resulting from dysfunction of the seventh cranial nerve were documented. DiGeorge syndrome and array-CGH deletions were excluded. On chest X-ray were signs of bronchitis and hyperperfusion of his right lung. An echocardiogram showed no shunts and no evidence of ventricular overload. A computed tomography (CT) angiography scan revealed an abnormal vessel arising from the thoracic descending aorta coursing toward his right upper lung. On endovascular occlusion, the angiogram confirmed the hyperperfusion of the right upper lobe. The diameter of the aberrant vessel was up to 8mm. No lung anomaly or other abnormal vessels were detected. Three-dimensional volume rendering of the malformation is shown in Fig.. Pulmonary hyperperfusion is a risk factor for recurrent cardiorespiratory infection and lung hypertension. This risk is probably higher in syndromic malformations. A mini-invasive vascular occlusion was attempted after a multidisciplinary evaluation (pediatric cardiologist, pediatrician, pediatric surgeon and interventional radiologist). Under general anesthesia, ultrasound-guided right femoral common artery access was gained. The major aortopulmonary collateral artery (MAPCA) was easily catheterized with a 4F vertebral Glidecath catheter. The hypertrophied pulmonary arteries of the superior lobe were opacified as well as the normal venous drainage excluding a lobar sequestration. Due to the very short length and ascending direction of MAPCA, coils as embolic agents were excluded because of the high risk of nontarget embolization. Through the Glidecath a 6mm bi-segmentary Amplatzer plug type IV was released but it appeared undersized and unstable. The plug was withdrawn from the outer portion. The Glidecath 4F was exchanged over a stiff wire with a 4F 55cm-long Cook sheath with its distal tip in the pulmonary tree. A new tree segments (or lobes) 8mm Amplatzer plug type II was released through the sheath inside the MAPCA. At completion angiography through the side arm of the sheath the MAPCA was embolized. Hemostasis was done at the femoral entry by manual compression. A chest radiogram and CT showed normalization of the vascular pattern of the right lung at 9-months follow-up. No complications and no respiratory infections in the first follow-up year were observed. A good growth gain was observed: weight 25kg, 75 percentile; height 121cm, 75 percentile.