Management of long standing left to right shunt lesion resulting in elevated pulmonary vascular resistance (PVR) is challenging. Limited surgical options are further complicated by an unpredictable postoperative period. Unidirectional valve patch (UVP) closure has shown to be useful in cases of the large ventricular septal defect (VSD) who present late. We report a case of large aortopulmonary window coexisting with a large VSD with severe pulmonary artery hypertension and significantly elevated PVR that was managed surgically by closure of the window by sandwich technique and closure of the septal defect with a UVP. This report emphasizes the importance of UVP in the management of such patients.