Aortic dissection in pediatrics is an extremely rare condition, which is generally related to predisposing factors such as connective tissue disorders, congenital heart disease and systemic arterial hypertension. A 3-year-old girl, with a history of bicuspid aortic valve, hypoplasia of the aortic arch and repaired aortic coarctation at one month of age. She was admitted 2 months of atypical chest pain, dysphonia, and low tone of voice. The echocardiogram and CT angiography showed an image corresponding to a Stanford A aortic dissection, with false lumen perfusion that generated a aneurysmal dilation with a saccular morphology of 53 × 40 × 70 mm dimensions. The patient was taken to surgery, exposure of the ascending aorta, aneurysmal dissection, and replacement with a 22 mm supracoronary tube were performed. We present a case of a 3-year-old pediatric patient with Stanford A aortic dissection, subacute evolution, with successful repair.