Two patients with D.O.L.V., V.S.D. and P.S. have been successfully corrected surgically. They represent the seventh and the eighth successful repairs of D.O.L.V. reported so far in the literature. Surgical correction was accomplished by an "extraventricular" type of repair, closing the V.S.D., interrupting the connection between left ventricle and pulmonary artery and creating a conduit between right ventricle and pulmonary artery. The conduit used was a fresh aortic homograft. An "intraventricular" type of repair of this rare congenital malformation has been also reported, but the authors believe that it is not applicable to all cases of D.O.L.V. and it is possibly more likely to incur complications, while the "extraventricular" one doesn't seem to have any contraindication. Preoperative diagnosis can be only achieved with careful angiographic study, including left ventriculography.