Abstract
We report a case of Uhl's anomaly in a 5-month-old cyanotic infant who presented with thromboembolic stroke and acute hemiparesis. The patient underwent successfully an initial surgical repair, which included exclusion of the right ventricle by patch closure of the tricuspid valve, atrial septectomy and construction of a bidirectional Glenn shunt. This was followed by successful construction of a total cavopulmonary connection.