Nine patients with complete atrioventricular canal and tetralogy of Fallot underwent intracardiac repair of both anomalies between 1982 and 1989. The ages of the patients ranged from 6 months to 7 years. Six of the 9 had one or more previous systemic-pulmonary artery shunts. Two-dimensional echocardiography showed diagnostic characteristics of both malformations in all patients. The diagnosis was confirmed by cardiac catheterization and cineangiography. The ventricular septal defect was repaired by a combined right atrial and ventricular approach in every patient. Outflow tract reconstruction was performed with the use of a transannular patch (4), infundibular patch (4), and a valved conduit (1). There were no hospital deaths. Meningitis was responsible for the death of a patient 2 years after repair. We recommend early palliation, complete repair in those older than 4 or 5 years, surgical technique depending on the anatomical findings, combined atrial and ventricular approach, 2 separate patches to close the ventricular septal defect in required cases, adjusted correction of the right ventricular outflow tract, and careful postoperative care.