A successfully corrected case of a 39-year-old female with viscero-atrial heterotaxic syndrome was reported. The cardiovascular anomalies were sinus venosus atrial septal defect, mitral cleft, and interruption of inferior vena cava with azygos continuation. She also had bilateral bilobed lungs, symmetric liver, right sided stomach, and malrotation of the bowel, indicating left isomerism. The atrial septal defect was closed with Teflon patch and mitral cleft was repaired with direct suture of the cleft. The postoperative course was uneventful and mitral regurgitation was trivial postoperatively.