Coronary artery (CA) anomalies are a group of congenital heart defects with a diverse clinical performance, from lifelong asymptomatic to severe consequences such as sudden cardiac death. In some cases, CA anomalies become an incidental finding during echocardiography. If there is a suspicion of CA anomaly, a radiographic investigation (computed tomography (CT) angiography or magnetic resonance imaging) should be performed to clarify the anatomy and indications for surgical correction.A case of diagnosing a tubular structure with hyperechoic walls in mitral valve projection during echocardiography is presented. The performed CT angiography confirmed the abnormal origin of circumflex artery from the right sinus of Valsalva with its retroaortic course. This echocardiographic sign is described in the English-language literature as Retroaortic Anomalous Coronary sign (RAC-sign)
