We report successful treatment of pulmonary valve agenesia with pulmonary stenosis and ventricular septal defect by infundibular resection, patch-closure of the VSD and implantation of a cryopreserved pulmonary homograft. Cases with mild to moderate hypoplasia of the pulmonary annulus are particularly well suited for direct pulmonary homograft implantation, while cases with severe hypoplasia and high right ventricular pressure after correction may be better treated with aortic homografts. Some technical aspects of homograft implantation (doing first the proximal suture, preserving the normal configuration of the valvular sinuses and using the subvalvular homograft tissue to enlarge the right ventricular outflow tract) may prevent significant valvular incompetence.