Introduction: Atrial Septal Defect (ASD) is one of the most common congenital heart diseases during childhood. Today, ASD closure is done by occlutech device via cardiac catheterization. ASD repair with transcatheter technique has shown high closure rate. However, coexistence of severe pulmonary stenosis and large defect in atrial septum is rare. When these two problems coexist, the result is inhibition of remarkable left-to-right shunt by obstruction of the RV output flow, which will preserve the pulmonary bed up to adulthood.
Methods: A 5-year-old girl with a history of exertional dyspnea and palpitation was referred for the treatment of severe valvar pulmonary stenosis and large ASD. Echocardiography showed a large secundum ASD (16 mm) with appropriate rims and also severe pulmonary valvular stenosis. The patient was candidate for closure of ASD by transcatheter procedure and balloon pulmonary valvuloplasty. 
Cardiac catheterization showed that the right ventricular pressure was 78 mm Hg and pressure gradient across the pulmonary valve was 62 mm Hg, also the ratio of pulmonary blood flow to systemic blood flow was 2.7/1. Anteroposterior and lateral views in the angiography of right ventricle showed severe valvular pulmonary stenosis and poststenotic dilation. Diameter of the ASD size by balloon was 16 mm. Pulmonary valve annulus diameter was 15-16 mm.
Results: The patient was picked up for closure of ASD by transcatheter procedure and balloon pulmonary valvuloplasty. There was no remaining shunt and complication on transthoracic echocardiography performed immediately and in later follow-ups.
Conclusion: Coexistence of severe pulmonary stenosis with a large defect in atrial septum is an uncommon event. Combined transcatheter closure of ASD and pulmonary balloon valvuloplasty is a practicable and effective therapeutic intervention for this problem which will bring satisfying outcomes.