Around 3-4 children born with congenital heart diseases have univentricular hearts, where the prognosis of univentricular hearts is poor in the past, with a survival rate of less than 50% during the first year and 10% during the first ten years of life. Based on a literature search, current advances in perioperative management of neonates with complex congenital heart diseases have increased their survival rate by 85%. To aid cardiothoracic surgeons worldwide, this comprehensive literature review will focus on the perioperative management of staged palliation surgery for functional univentricular hearts, considering current trends as well as how we do it in our centre. Our review article specifially discusses perioperative strategies regarding surgical considerations, current techniques, to deal with overshunting and undershunting during the first stage of palliation surgery. This article also gives an overview on when a patient is suitable to go through with the next stage of the procedure, which is the implementation of a bidirectional cavopulmonary shunt or the Hemi-Fontan procedure. Lastly, this article gives a comprehensive approach regarding perioperative strategies of the Fontan procedure, which include patient criteria, current surgical techniques, postoperative management, as well as the use of anticoagulants after the Fontan procedure.