We report a long-term survivor with Uhl's anomaly who underwent one and a half ventricle repair combined with a partial right ventriculectomy in infancy, followed by successful total cavopulmonary conversion with right ventricular exclusion 5 years later. The combination of total cavopulmonary connection and right ventricular exclusion could be the optimal surgical option for a critically ill infant with Uhl's anomaly.
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