Introduction
Coronary artery anomalies are relatively uncommon congenital disorders of the coronary artery anatomy. The incidence of coronary artery anomalies has been reported to be 0.6–1.3%. [1] Left main coronary artery atresia (LMCAA) is the rarest form of congenital coronary malformations, in which coronary ostium and main trunk in the left coronary artery system is absent [2]. Thus, blood flows from the right coronary artery to the left coronary artery through collateral vessels [3]. Clinical presentation, management, and prognosis of this disease depend on the characteristics of the collaterals and native vessels [4]. Hereby, we report a case of congenital atresia of the left main coronary artery, misdiagnosed as Anomalous left coronary artery from the pulmonary artery (ALCAPA).