Aortico-left ventricular tunnel is a rare entity resulting in a communication between the ascending aorta and the left ventricle, with clinical signs of aortic incompetence. On the basis of two personal cases and a review of the literature including 57 patients, the operative mortality and long-term follow-up of these patients are analysed. 51 patients were operated upon with a mortality rate of 21.5% (11 cases). In this mortality group, 64% of the casualties were less than 2 years' old (7/11). Mortality was unrelated to the surgical technique. Aortic incompetence was the crucial factor of post-operative morbidity. 60% of the patients who were operated upon had significant post-operative aortic incompetence. Among the 21 patients with long-term follow-up (1.5 to 14 years), 17 have aortic incompetence (81%), 8 were reoperated and 7 underwent aortic valve replacement. Surgical repair of this malformation should be considered early in life, and the technique used should aim at consolidating the aortic annulus without deformation and at closing the aortico-ventricular window.