The association of a restrictive ventricular septal defect with a pulmonary tract obstruction is rare and may cause diagnostic and therapeutic problems. The authors report their experience of such an association in 10 children. 3 had a tetralogy of Fallot (1 death), 4 a double-outlet right ventricle (2 deaths), and 3 a coarctation of the aorta with ventricular septal defect and pulmonary artery banding (no death). Prognosis depends on the type of malformation. It has improved with years because of sooner recognition of these malformations and better surgical technics and post-operative care.