The criss-cross heart is a rare anomaly in which systemic and pulmonary bloodstreams cross at the atrioventricular (AV) level. Although diagnosed now with increasing frequency, successful surgical repair of associated anomalies has been reported only once. Straddling AV valve is another rare cardiac anomaly which only recently is being diagnosed consistently preoperatively and corrected surgically. This is the first report of successful repair of cardiac anomalies in a patient with the combination of straddling AV valve and criss-cross AV relationships. The uneventful postoperative course and good result on follow-up in this patient are gratifying and suggest that this combination of anomalies now can be repaired with results as good as those for less complex forms of congenital heart disease.