Aortico-left ventricular tunnel (ALVT) is an extremely rare, abnormal paravalvular communication between the aorta and the left ventricle. Few studies have identified the characteristics and long-term prognosis associated with ALVT. The data of 31 patients with ALVT from July 2002 to December 2019 were reviewed. Echocardiography was performed in all patients during the follow-up period. The median age of the patients was 11.5 years. Bicuspid aortic valve and dilatation of the ascending aorta were found in 13 patients, respectively. The aortic orifice in 20 patients showed a close relation to the right sinus and the right-left commissure. Of the 31 patients, 26 were operated on. Mechanical valve replacement was performed in 4 patients and aortic valve repair, in 6 patients. Ascending aortoplasty was performed in 5 patients and aortic replacement was done in 2 patients. One patient died of ventricular fibrillation before the operation. Follow-up of the remaining 30 patients ranged from 1 to 210 months (median 64 months). There were 4 deaths during the follow-up period: 1 had mechanical valve replacement and 3 did not undergo surgical repair. In the 26 patients without aortic valve replacement, 6 had severe regurgitation and 2 had moderate regurgitation. In the 28 patients without replacement of the ascending aorta, 11 had continued dilatation of the ascending aorta, including those who had aortoplasty. The aortic orifice of ALVT showed an association with the right sinus and the right-left commissure. For patients who did not have surgery, the long-term survival rate remained terrible. Surgical closure should be done as soon as possible after ALVT is diagnosed. The main long-term complications after surgical repair included aortic regurgitation and ascending aortic dilatation.