This is a collective review of aortico-left ventricular tunnel (ALVT) in the English-language literature. We include the long-term follow-up of a previously reported patient, and a report on 2 new patients. To date, 37 cases of ALVT have been reported. Controversies regarding the definition, etiology, local anatomy, and treatment are discussed. The ages of the patients ranged from 1 day to 25 years old, and the male to female ratio was 2:1. There were associated anomalies in 27% of the patients, and moderate to severe heart failure in 59% of them. Mortality was 100% in the medically managed group; the surgical mortality was 16%. Previous surgical techniques utilized were simple closure, patch closure of the aortic end, and obliteration of the tunnel on both ends. Progressive aortic incompetence seems to be a common, but not well-documented problem on long-term follow-up. We have classified the lesion into four types (I, II, III, and IV) that have a bearing on the appropriate surgical techniques of repair, and describe a new technique for the repair of type III ALVT in which septal aneurysm is present.