Tetralogy of Fallot is the most common form of cyanotic congenital heart disease and accounts for 10% of all congenital heart defects. Corrective surgery has dramatically improved long-term prognosis, with nearly 90% of patients now surviving well into adulthood. Aortic root dilation is a well-recognized feature of unrepaired tetralogy of Fallot, and is increasingly reported in patients, years after corrective surgery. While the prevalence and severity of aortic root dilation and its associated complications remain to be defined, a clearer portrait is emerging from the growing body of literature. The objective of this contemporary review is to address the scope of the problem regarding aortic dilation in tetralogy of Fallot, explore potential pathophysiological mechanisms, summarize current knowledge regarding adverse events and discuss potential therapeutic options.