Background. Tricuspid valve insufficiency secondary to structural anomalies of the valve itself or to an iatrogenic complication of the Rashkind procedure is very rarely associated with transposition of the great arteries. This condition represents an interesting perioperative challenge. Rapid restoration of the tricuspid valve to a low-pressure system by arterial switch operation associated with tricuspid repair should theoretically improve the outcome in terms of myocardial and valve function. Methods. Thirteen of 839 patients who underwent an arterial switch operation for various forms of transposition of the great arteries presented with moderate to severe tricuspid insufficiency. Three of them had a ventricular septum defect. Nine experienced severe cardiac failure with profound hypoxemia. Ventilatory support was necessary in 7, 6 had renal or hepatic dysfunction, and 5 had coagulation disorders. Inotropic support was started preoperatively in 8 patients. Results. Tricuspid lesions were as follows: primary annular dilatation and lack of coaptation at the commissural level (n = 1), straddling tricuspid valve (n = 1), redundant tricuspid valve tissue leading to left ventricular outflow tract obstruction (n = 1), small cleft of the septal leaflet (n = 1), and dysplastic valve tissue with juxtacommissural regurgitation (n = 1). In 8 patients, the cause of the tricuspid valve insufficiency was most probably an iatrogenic lesion, with rupture of the papillary muscle (n = 2), rupture of the chordae (n = 1), or tear of the anterior leaflet (n = 5), whereas no clear cause could be found in 1 patient. Repair consisted of the arterial switch operation associated with tricuspid valve repair in 10 patients. In 2 patients with only discrete anomaly and in 1 without a clear cause of tricuspid regurgitation, no valve repair was performed. Three patients had their ventricular septal defect closed. There were one early and one late death, both not related to the tricuspid lesions. Late postoperative (mean, 6.5 years) evaluation revealed normal left ventricular function in 10, with no tricuspid incompetence in 7 and trivial tricuspid insufficiency in 3. Conclusions. Restoration of an incompetent tricuspid valve in a low-pressure system by the arterial switch operation combined with valve repair provides good ventricular and valvar results. Preoperative management and appropriate timing of operation seem to be of utmost importance.