Between June 1989 and September 1991, 11 patients underwent anatomic correction of atrioventricular discordance. Their ages at operation ranged from 1 to 11 years (mean 6.7 years) and their weights ranged from 7.1 to 31.8 kg (mean 19.1 kg). Atrial situs was solitus in nine and inversus in two patients. Ventriculoarterial connection was discordant in five and was double-outlet right ventricle in six patients. Associated congenital heart defects were seen in all patients, including 10 with ventricular septal defect, eight with atrial septal defect, nine with pulmonary stenosis or pulmonary atresia, seven with tricuspid regurgitation, and four with mitral regurgitation. Five patients had prior Blalock-Taussig shunts. One patient with an intact ventricular septum had repeated pulmonary banding. Anatomic correction consisted of the Senning and Rastelli procedures in three, the Mustard and Rastelli procedures in five, the Senning and arterial switch operations in two, and the Mustard and arterial switch operations in one patient. In addition, mitral valvuloplasty or valvular annuloplasty was performed in three patients. We did not encounter kinking or torsion of the translocated coronary arteries in our three patients with the arterial switch operation. There was one surgical death. The other patients pursued satisfactory postoperative courses (mean follow-up period of 12.6 months). We recommend that anatomic correction for atrioventricular discordance should be indicated, especially in patients with any sign of systemic right ventricular dysfunction.