Anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva is a relatively rare congenital cardiac defect. While the majority of these anomalies are benign, there are certain coronary anomalies that increase the risk of sudden cardiac death in the young. Patients may present with cardiovascular complaints while others may be asymptomatic at diagnosis. Transthoracic echocardiography is useful for the initial diagnosis. Often, further non-invasive imaging, such as cardiac MRI or CT scan, is used to confirm and further delineate the coronary anatomy. Treatment is dependent on the anomalous coronary artery course as well as patient presentation. There is controversy regarding the best treatment for those anomalous coronary arteries that course between the aorta and pulmonary artery, especially in the asymptomatic patient with anomalous right coronary artery from the left sinus of Valsalva. Future directions should focus on better methods to risk stratify these pediatric patients.