Concomitant presence of acute type A dissection and coactation of aorta is rare (1). Levoatriocardinal vein has shown to be associated with left sided hypoplastic lesions as well as with normal hearts (2, 3 ). However, concurrent presence of levoatriocardinal vein with acute type A dissection, severe aortic regurgitation and Coarctation of aortic isthmus was not described. We here described a case of 20 year male presented to emergency department with acute chest pain radiating to back. On evaluation, he was found to have acute type A dissection with dilated aortic root, severe aortic regurgitation, normal mitral valve, severe coarctation of aorta and levoatriocardinal vein. Patient was managed successfully with composite valve conduit replacement of ascending aorta with ascending aortic to descending aortic graft (16mm graft) with levoatriocardinal vein ligation.