Double inlet left ventricle (DILV) is a complex cyanotic lesion with several pathophysiological components. Both atrioventricular valves empty into one ventricle, with the other ventricle rudimentary and connected by a bulbo-ventricular foramen. Most cases are associated with additional cardiac defects, most commonly with a transposition of the great arteries or pulmonary stenosis or atresia. Anaesthetic management depends on the individual lesions and their severity. This is a mixing lesion with varying degrees and locations of obstruction, resulting in either a decreased systemic or decreased pulmonary blood flow.